Central neurocytoma is a rare, well-differentiated neuronal tumor and is usually located in the lateral or third ventricle of young adults. Its overall prognosis is excellent with a low proliferative index. The majority of previously reported malignant variants rarely did recurred after tumor removal and regarded as benign tumor despite of histopathological malignant feature. Nevertheless, we experienced a case of malignant variant of the central neurocytoma with high proliferative index(Ki-67 labeling index >30%), which showed recurrence immediately after surgery and died within 3 months POD. Here, we describe the case with a review of the literatures.