Cerebellar liponeurocytoma is a rare WHO grade II glioneuronal tumor, characterized by a benign course and a more favorable prognosis. In this paper, we report a 52-year-old man, hospitalized in emergency because of clinical signs of increased intracranial pressure, and gait disturbances. The MRI revealed a lateral well shaped solid lesion within the left cerebellar hemisphere, having a low signal intensity compared to the cerebrospinal fluid, and causing a triventicular hydrocephalus and tonsillar herniation. The patient underwent a direct approach of the tumor through a left suboccipital craniectomy. Total removal of the lesion was achieved, and the histological diagnosis was cerebellar liponeurocytoma. The tumor cell proliferation index was < 6 %, therefore, radiotherapy and chemotherapy were not implemented after surgery. After 5 years of close follow-up there were no clinical or radiological signs of recurrence. Our objective in reporting another case of this unusual tumor is to discuss clinical profile, radiologic and histologic features as well as treatment and prognosis of this rare posterior fossa tumor.