Sturge-Weber syndrome (SWS), also known as encephalofacial angiomatosis or encephalotrigeminal angiomatosis, is a rare congenital neurocutaneous syndrome characterized by facial and intracranial angiomatosis. Sturge and Weber reported cases in 1879 and 1922, and the name Sturge-Weber syndrome was first used and named by Bergstrand in 1936. However, since the incidence of SWS is only 0.002% or less, there has been no uniform standard for its diagnosis and treatment. In particular, surgical treatment has rarely been reported in China. In this paper, we report the clinical data of a patient treated in our department to discuss the preoperative evaluation and surgical methods for the surgical treatment of SWS. The patient, male, 24 years old, had his first seizure at 12 d of birth, which was characterized by tonic jerking of the extremities, upturning of both eyes, head deviation to the left, no cyanosis of the lips and mouth, and the seizure stopped after 2-3 s. The patient had a seizure for about 1 to 2 months afterwards. He was given carbamazepine 0.05g and sodium valproate 0.2g orally, but the frequency of seizures did not decrease significantly, and he discontinued the medication after 3 months. Now he felt palpitations and discomfort before the seizure, and the left limb twitched first during the seizure, then the whole body twitched, and occasionally he talked nonsense after the seizure and scratched his hands on his body, and the seizure stopped after a few minutes. On examination, he saw irregularly shaped red hemangiomas on the skin of the right frontal, lateral orbital and zygomatic areas, flattened on the skin surface and discolored by pressure. There was no intraocular vascular nevus and normal intraocular pressure. There were no positive neurological signs. Wechsler’s intelligence was low to moderate. MRI showed calcification of the brain surface of the right temporal and occipital lobes with localized brain atrophy and bilateral hippocampal atrophy and sclerosis (. Spectral analysis suggested that the N-acetyl aspartate (NAA) peak was decreased and the choline (Cho) peak was increased in the hippocampus bilaterally. The video EEG showed reduced electrical activity on the right side during the interictal period, and the right temporal, parietal, and occipital areas were dominated by single 2c/s spikes and slow waves with amplitudes of 80-100μν. The EEG showed muscle tonic waves during the seizure, and no obvious pre- and post-ictal discharges were observed. Visual field examination suggested bilateral left-sided isotropic hemianopia. Past history and family history were negative. A combined incision was made in the right frontotemporal-occipital region. Intraoperatively, the meningeal surface was rich in blood supply, and the right temporal and occipital lobes were dark purple with abnormal reticular vessels and cortical calcification on the surface. Cortical EEG (electrocorticography, EcoG) traced the abnormal slow waves in the temporal and occipital lobes and high amplitude spikes in the right central region. 3cm and 5cm posterior to the temporal pole in the right middle temporal gyrus plane, the deep electrodes were perpendicularly punctured 3.5cm, and the amygdala and hippocampus EEG were measured. The right anterior temporal lobe, hippocampus and amygdala were excised, and the abnormal cortices of the right posterior temporal and occipital lobes were excised. The size of the abnormal brain tissue was about 8cm×6cm×3cm, the hippocampus was about 2cm×1.2cm×2cm, and the amygdala was about the size of a rice grain. After resection, EcoG tracing was performed, and the EEG wave amplitude was reduced at the resected area, and no spike or slow wave was seen. The patient continued to take Depakene after the operation and no seizures were observed during the hospitalization. The EEG was repeated and only a small number of 10c/s alpha waves, 25-50 μν, were seen, with reduced electrical activity on the right side. Pathology showed marked congestion on the surface of the resected brain tissue, and numerous proliferating abnormal vessels were visible. The gray matter was atrophied to varying degrees and contained gravel bodies of varying sizes. Light microscopy showed a decrease in neurons with degeneration and proliferation of glial cells in the white matter. Neuronal degeneration and atrophy were seen in the hippocampus and amygdala, and gravel bodies were seen in the hippocampus. Postoperative CT was repeated and the calcified lesion was completely excised. No seizures were seen at the 3-month postoperative follow-up. The typical clinical manifestations of Sturge-Weber syndrome are a cutaneous hemangioma on one side of the face (often in the area of the first and second branches of the trigeminal nerve), epilepsy and ipsilateral glaucoma, while one or two may be missing in atypical cases. The etiology of the disease is still unclear. It is thought to be caused by abnormal development of neuroectoderm and vascular mesoderm at 4~8 weeks of embryonic life, but recent studies have found that mutations in the RASA1 gene may be closely related to SWS. In combination with the fact that most of the previous cases were disseminated, the syndrome is now considered to be a congenital disease rather than a genetic disease. The current treatment for SWS is mainly symptomatic, while the focus of epilepsy treatment is to reduce seizures and avoid further worsening of brain damage due to recurrent seizures. Anti-epileptic drug therapy is generally preferred to carbamazepine, and valproic acid and phenytoin sodium can also be chosen, but all have poor effects. Surgical treatment has gradually been accepted, but the timing of surgery is inconclusive. Some scholars believe that surgery should only be performed after long-term formal antiepileptic treatment has failed. The results of some studies suggest that surgery in infancy (up to 2 years of age) may not only abort seizures but also improve the quality of survival by avoiding further neurological damage caused by epilepsy, but the results remain to be further validated. Early surgical treatment of this syndrome is still preferred, and the commonly used procedures include cortical resection, lobectomy, hemispherectomy, and corpus callosotomy. It has been previously reported that total excision of the diseased cortex or lobes is effective in controlling epilepsy. In patients with extensive hemispheric damage with refractory epilepsy, hemispherectomy may be more effective. Previous case reports suggest that patients who underwent hemispherectomy in infancy had good postoperative seizure control and almost complete recovery of motor deficits that occurred after surgery, which may be related to the greater neurological compensatory function of infants, whereas older patients who underwent hemispherectomy had permanent motor deficits to varying degrees. The corpus callosotomy is a palliative procedure and can be considered only when the patient has frequent fall episodes or when no other surgical modality can be performed. There are few case reports about this procedure, which only suggest that the short-term results are still possible and the long-term results are uncertain. In this case, MRI and spectral analysis suggested that the anatomical lesion was located in the right hemisphere, with sclerosis in the hippocampus, and the EEG showed reduced right-sided extensive electrical activity and paroxysmal spikes and slow waves in the parieto-occipital region. Considering the possibility of more severe motor and cognitive dysfunction after hemisphere resection, we chose lobectomy and removed the calcified right temporal and occipital cortices, as well as the ipsilateral hippocampus and amygdala under EcoG supervision. Intraoperatively, slow waves were found to be present in the peripheral cortex of the lesion, while high amplitude spike waves were issued in the central region of the distal lesion site, although there were no obvious pathological changes. This is somewhat different from the results of Rasmussen [8], who found that the most active epileptogenic foci were generally located in the peripheral cortex of the angiosarcoma lesion. The mechanism of the abnormal discharges in the central region, consistent with the spike-like waves in the parietal region seen on preoperative EEG, is less clear, and could be due to a soft membrane vasculopathy affecting the blood supply to the area or secondary brain damage caused by recurrent seizures. However, the high amplitude spike-wave distribution in the functional cortex may be closely related to the frequent seizures of the patient, suggesting that the epileptic focus and the anatomical lesion may not be completely consistent, which poses a higher requirement for the preoperative localization of the epileptic focus. Since SWS is mostly disseminated and cases are rare, there are still many issues for the surgical treatment of this syndrome that deserve further discussion, such as the selection of surgical approach and timing, preoperative patient assessment, the impact on motor and cognitive functions after surgical treatment, and the use of postoperative antiepileptic drugs.