A 29-year-old male patient with von Hippel-Lindau disease who had an operation for cerebellar hemangioblastoma six years ago is presented. The patient had a four-year history of a slow-growing, locally invasive vascularized lesion of the temporal bone involving the cerebellopontine angle. The mass, studied by MR imaging, was almost completely cystic in appearance. Pathological examination after removal of the mass confirmed a papillary endolymphatic sac tumor.

Keywords: magnetic resonance imaging, von Hippel-Lindau disease, ear, inner, neoplasm