Central neurocytoma (CN) is a rare intraventricular brain tumor that affects young adults. Just over 100 cases of CN have been reported since it was first described in 1982. It accounts for less than 1% of all central nervous system neoplasms. It is characterized by cystic spaces and intratumoral calcification. The tumor is generally hypervascular. Patients generally present with signs and symptoms of noncommunicating hydrocephalus such as headache, visual disturbances such as blurry vision and visual field deficit, and nausea or vomiting or both. Gross total surgical resection is the treatment of choice for CN. Other treatment modalities such as radiation therapy, radiosurgery, and chemotherapy may offer adjunctive or alternative treatment for residual or recurrent CN. The clinical outcome is generally good.