OBJECTIVE:: Neurocytomas (NCs) comprise a rare but distinct clinicopathologic entity, often with a benign course. Our operative series of 33 patients with NCs attempts to review the clinical characteristics of this rare entity. METHOD:: Retrospective analysis of the case records of all the patients with central NCs operated since January 2000. The following prognostic factors were analyzed for statistical significance: age, sex, location, extent of resection, and the histopathologic grade. RESULTS:: The study group included 33 patients with a mean age of 28.87 years (range, 9 to 55 y) and with a male preference (M:F=20:13). Increased intracranial headache was the predominant presenting complaint (27/33; 81.8%), followed by gait imbalance, memory deficits, weakness, and seizures. At the first surgery, gross total removal could be achieved only in 16 cases (48.84%). The majority (26/33; 78.78%) were typical NCs and 7 were atypical NCs. Of the 17 patients with residual lesions, only 4 patients showed an increase in the size of the residue and only 2 of them had symptomatic recurrence requiring repeat surgery. Of the 16 patients who underwent radical excision, 4 had recurrence, 1 requiring repeat surgery. Three of the atypical NCs recurred in spite of gross total resection. We had 5 perioperative mortalities, all related to tumor bleeding and postoperative brain swelling. One child with a recurrent fourth ventricular NC expired after adjuvant chemotherapy. Of the remaining 27 patients, 25 patients had a Glasgow outcome score (GOS) of 5 and 2 were in GOS 3 at the end of a mean follow-up period of 4.63 years (range 2 to 11 y). CONCLUSIONS:: Central NCs are unique tumors of neuronal origin with strong histologic resemblance to other intraventricular tumors of glial origin. NCs are vascular tumors, and their radical removal can be challenging. Although radical surgery is the treatment of choice, they are slow growing, and subtotal resections also provide long-term symptom-free survival even for atypical lesions. Copyright © 2012 by Lippincott Williams & Wilkins.