Objective: To investigate the clinicopathological features, diagnosis and differential diagnosis, and prognosis of central neurocytoma (CNC). Methods: The clinical manifestations of 13 cases of CNC were analyzed retrospectively and the literature were reviewed. Results: All the tumors in 13 patients were in the brain chamber, including 7 males and 6 females. The age of onset was 18–60 (median 29) years old. Dizziness and headache were the most commonly presenting symptoms. CT showed equal density or slightly higher density, with 7 cases of calcification, 8 cases of cystic change, mild enhancement after enhancement; MRI showed T1 low signal, slightly T2 high signal or high and low mixed signal, light and moderate reinforcement of the solid part after reinforcement. Histologically, the tumor tissue consisted of a single round cell arranged in a honeycomb shape, the blood vessels were branched, 1 case showed a slightly active mitotic division, 1 case showed focal necrosis; immunohistochemistry showed that Syn, NSE and NeuN of tumor cells were positive, while IDH-1, Olig-2 and EMA were negative, GFAP and S100 were focally positive. 11 cases had a Ki-67 index <2%, and 2 cases had a Ki-67 index 5%~10%. All 13 patients underwent microsurgical tumor resection. Six patients underwent postoperative radiotherapy, followed up for 8 months to 5 years, and 1 case relapsed. Conclusion: CNC is a rare central nervous system tumor with good prognosis. The diagnosis needs to be combined with lesions, histological features and immunological markers. Total surgical resection is the best way to treat CNC, post-operative combined radiotherapy and chemotherapy can reduce the recurrence rate, but the effect also needs to be further studied.