Gliomas are prone to seizures, especially low-grade gliomas, often with epilepsy as the first symptom. Complete removal of the tumor and removal of the tumor peripheral tissue based on intraoperative EEG can provide excellent seizure control, even to the point of being seizure-free for life. Common low-grade gliomas that cause seizures include ganglion cell tumors and ganglion cell gliomas. Ganglion cell gliomas were proposed by Courville in 1930 and account for 0.4% to 1.3% of all brain tumors and 10% of central nervous system tumors in children. Seizures in patients can be the only clinical manifestation due to chronic irritation and compression of the cortex by the tumor creating epileptic foci around the tumor. It is the most common cause of epilepsy in tumorigenic lesions. The histogenesis of ganglioglioma/ganglioglioma is not well understood. It may originate from embryonic remnants of ganglion cell precursor cells, or it may be associated with local developmental abnormalities or malformed tumorigenic lesions. Recent studies have found that patients with gangliogliomas often have chromosome 9 and 7 abnormalities. They are purely neuronal tumors, mainly composed of ganglion cells, with a few spindle cells (spindle glial cells) and blood vessels as interstitial, without glial cell component or malignant tendency. Clinical manifestations The tumor is usually found in the temporal lobe, followed by the base of the third ventricle, subthalamic and thalamus. 70% or more of patients have epilepsy as the main clinical symptom, and headache and mental disorder may also appear. The disease progresses slowly, often up to 4~5 years from the first symptoms to a definite diagnosis. The tumor may be high-density on CT scan or without abnormal changes, low-signal on T1WI, equal or slightly high signal on proton density-weighted image or T2WI, non-enhanced or mildly enhanced on enhanced scan, and mostly solid. Magnetic resonance spectroscopy (MRS) mostly suggests slightly decreased NAA and significantly increased Cho. Intraoperative cortical EEG shows epileptiform discharges located in or around the lesion. Pathology According to the various editions of WHO classification, gangliogliomas are classified as subtype neuronal and mixed neuronal-glial tumors under the category of neuroepithelial tumors (WHO classification I-II), which are slow growing and benign. The case picture, under light microscopy, consists of mature ganglion cells and protrusions with irregular distribution of ganglion cells, single, double or multiple nuclei, seen with nucleoli, intracytoplasmic niche, and tumor tissue mixed with myelinated and unmyelinated nerve fibers. In some cases, there are a certain number of glial cells in the tumor tissue, which is called ganglion cell glioma, and if the glial cells are heterogeneous, it is called mesenchymal ganglion cell glioma. Immunohistochemically, the tumor tissue is positive for GFAP markers in glial cells and positive for NF, NSE, Syn and CgA markers in ganglion cells. Electron microscopy revealed granules, presynaptic vesicles, and synaptic structures in the tumor cells. Treatment The literature reports that surgical excision of ganglion cell gliomas provides excellent control of patients’ seizure symptoms, with 63%-100% of patients being seizure-free after surgery. Therefore, in patients with low-grade glioma with epilepsy, once the tumor is clearly identified as the epileptogenic factor, it should be surgically removed as early as possible to relieve the compression of the tumor on the surrounding normal brain tissue and eliminate the epileptic foci, and the adverse effects of long-term seizures on patients. Author’s practical experience Our functional group has completed nearly 80 cases of low-grade glioma in the past 10 years under intraoperative magnetic resonance and intraoperative electrophysiological monitoring, ranging in age from 7 months to 60 years, most of which were located in non-functional areas of the frontal temporal lobe, with a very high postoperative seizure-free rate of 90-100%, and only less than 10% of patients with incomplete surgical resection because the lesions were located in functional areas and functional impairment might occur after surgery. Postoperatively, epilepsy is significantly reduced, but not cured.