A 36-year-old female presented with vertigo and worsening of a right hearing loss with tinnitus. Clinical and radiologic investigations revealed a tumor of the right internal acoustic meatus, first diagnosed as a neuroma. Histologic study of surgical samples led to a final diagnosis of lipoma. Cranial nerve lipomas are rare and usually located in the cerebellopontine angle. Lipomas confined to the internal acoustic meatus are much rarer. The review of the literature however, shows that their histologic characteristics and their behavior are identical to those of cerebellopontine angle lipomas, and that a minimal surgical resection is therefore advised.