To discuss the clinicopathological features of liponeurocytoma, we retrospectively reviewed three liponeurocytoma cases and compared their immunophenotypes and genotypes with those of similar tumors. Furthermore, we reviewed the literature and compared the similarities and differences between cerebellar and intraventricular liponeurocytomas. Two cerebellar and one intraventricular liponeurocytomas were included in the present study. The liponeurocytomas comprised small tumor cells and lipomatous cells. The tumor cells expressed SYN, MAP-2, and NeuN. One case showed atypical histological features. By reviewing the literature, we found that cerebellar liponeurocytoma tended to be more common in females, whereas the converse was true for intraventricular liponeurocytoma. Compared with cerebellar liponeurocytoma, intraventricular liponeurocytoma was more commonly noted in younger adult patients. A high MIB-1 index (>10%) and incomplete tumor resection might represent adverse prognostic factors in patients with liponeurocytoma. We suggest that 'central liponeurocytoma' should be used to include all putative liponeurocytoma sites. The present study identified several morphological, immunohistochemical, and genetic features that may aid in the differential diagnosis of liponeurocytoma. In addition, surgery should be the preferred treatment, and complete tumor resection should be the goal. Additional cases with long-term follow-up are needed to develop optimal management protocols for liponeurocytoma.