Introduction Cerebellar liponeurocytoma is a rare tumor of the central nervous system occurring mainly in the posterior fossa, which shows neuronal and variable astrocytic differentiation, along with foci of lipomatous differentiation. Case presentation Herein, we describe a 50-year-old female patient who presented to the hospital complaining of headache, tinnitus, and vertigo with positive cerebellar signs. MRI revealed a left cerebellar tumor. After tumor resection, histological examination and immunohistochemistry were done and the diagnosis of cerebellar liponeurocytoma was confirmed. Discussion Liponeurocytoma may be mistaken as a medulloblastoma with lipidized cells or a lipomatous ependymoma. Histopathological examination, reinforced by immunohistochemistry and electron microscopy, are required to distinguish between these entities. The rarity of this tumor and paucity of pertinent information regarding its biological potential and natural history have resulted in the application of various treatment modalities. Conclusion Liponeurocytoma is a rare benign tumor with cerebellum is the typical site for it. Although surgery is the treatment of choice; however, postoperative radiotherapy may have a role in case of incomplete tumor resection or recurrence., Highlights • Cerebellar liponeurocytoma is a rare benign tumor that mainly occurs in the posterior fossa. • Surgery is the first line in management. • Postoperative radiotherapy has a role in case of incomplete tumor resection or recurrence.