We report a unique case of primary multiple liponeurocytoma. Liponeurocytoma is a rare benign tumor (Grade II) of the posterior cranial fossa with neural- or probably astrocytic-lineage cells; the tumor stroma contains mature adipocytes. This case is the thirty-eighth case of this pathology reported in the world literature and the first case of multiple form of this disease. We have provided a sufficiently thorough neuroradiological and histological picture that allows one to differentiate between a liponeurocytoma and other histological variants. Recommendations for the tactics for managing patients with this rare disease are proposed based on the analysis of treatment of this pathology reported in the world literature.