I would like to discuss my experience with the treatment of atlanto-occipital deformity combined with spinal cord cavitation. Usually such patients often do not care when they first develop sensory disturbances, and among my many patients, most of them choose acupuncture or opioid symptomatic treatment in Chinese medicine, thus making the condition gradually deteriorate in the process of further masking the condition, and there are more patients in rural areas, which is related to the economic development between cities and the popularity of medical knowledge, leading to delays. Most of the patients started to pay attention only after they developed temperature perception disorder, causing large burns on the back of the arm or shoulder. Most of the patients start to pay attention to the condition only after the development of temperature perception disorder, which causes large burns on the arm or shoulder and back, but they consider the “marginal” department when they seek medical treatment, and finally go to the neurosurgery department for cranial MRI, which leads to misunderstanding of the disease. The presence of atlanto-occipital deformity was found only after further 3D reconstruction of the cervico-occipital junction area, which includes more categories: cervico-occipital fusion; atlanto-axial fusion, skull base flattening and depression; dentate entrapment and so on. For neurosurgeons, we mostly use posterior cranial fossa decompression for spinal cord cavity surgery, which includes many ways: simply removing the posterior occipital to posterior occipital foramen bone flap without cutting the dura; removing the bone flap and cutting the dura at the same time and expanding the dura; performing cerebellar tonsillectomy at the same time. The results may vary, with some imaging improving but the patient’s symptoms persisting, and some imaging not relieving but the patient’s symptoms doing decrease. Patients with atlanto-occipital deformity often have cervico-occipital instability after posterior cranial fossa decompression. It is concluded that our department combined with orthopedics to perform anterior dentate grinding and posterior cervico-occipital fusion with internal fixation, which largely avoided the complications caused by patients with postoperative instability in the cervico-occipital junction region. And the clinical results are remarkable. After summing up the experience, it is necessary to perform 2D and 3D reconstruction of the craniocervical junction area for each patient before surgery, so that we can have a better understanding of this area before surgery and avoid important nerve and vascular tissues in this area while effective internal fixation, which can guarantee the smooth operation. At the same time, the most serious problem for transoral dentate removal is cerebrospinal fluid leakage, so it is crucial to operate slowly with a miniature grinding drill.