A case of cerebellar lipomatous medulloblastoma in a 55-year-old male subject, clinically presenting with signs of intracranial hemorrhage and cerebellar dysfunction, is described. Magnetic resonance imaging revealed an enhancing irregular vermian lesion invading the left cerebellar hemisphere. The patient underwent a craniotomy with total excision of tumor. Histologic examination revealed that a large portion of the tumor was composed of mature adipocytes. Lipoblasts with multiple vacuoles were not detected. Remaining tumor exhibited morphological, ultrastructural, and immunohistochemical features of medulloblastoma with both neuronal and glial differentiation. DNA analysis by flow cytometry revealed that the neoplasm was aneuploid. After 8 months, a new magnetic resonance imaging did not show any recurrence and the patient was free of symptoms. Morphological and immunohistochemical features, together with the age at the time of surgery and DNA content, suggest that lipomatous medulloblastoma is to be considered as a rare but distinct variant of medulloblastoma.