Intracranial lipomas are uncommon congenital malformations that are often asymptomatic. They are very rarely seen in the inner ear. There are a few case reports in the literature related to intravestibular lipoma. It was also defined in the internal auditory canal and the cerebellopontine angle. We present here a case of an inner ear lipoma that diffusely infiltrated the cochlea, vestibule and the semicircular canals. To our knowledge, this is the first reported case in the literature.