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Central neurocytomas are rare, mostly benign neuroectodermal tumors of the central nervous system typically located within the lateral and third ventricles of cerebrum. No consensus guidelines for the management of central neurocytoma available due to the rarity of the disease. We report a case of right ventricular central neurocytoma of a 28-year-old lady who had a subtotal resection and ventriculoperitoneal shunting. Postoperatively, she was treated with concomitant chemotherapy with oral temozolomide and radiotherapy, followed by add