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Abstract Background: Liponeurocytoma is a rare tumor of the central nervous system(CNS) characterized by low neuronal proliferation with foci of lipomatous differentiation. to the best of the authors` knowledge, 70 cases of brain liponeurocytoma have been reported which most of them are located in the cerebellar hemisphere but only 7 case of   primary cerebellopontine angle liponeurocytoma have been reported till now. Herein we presented a 27 -year-old woman presented to our clinic with a 6-month history of progressive headaches, dizziness.  More recently, he developed progressive hearing loss and   ataxia. Pre-operative Magnetic resonance imaging (MRI) of the brain demonstrated a heterogeneous solid extra-axial lesion, in the right cerebellopontine angle with hydrocephalus. The operation was performed via a right  retro sigmoid approach. The final histopathological diagnosis was confirmed as liponeurocytoma. Radiation therapy was not done and close observation was maintained during the follow-up for the subsequent 2 years; no signs of recurrence were found.