A 68-year old man with a cerebellopontine angle tumor.

We report on a 68-year-old male with a cerebellopontine angle tumor manifesting at the 8th cranial nerve and presenting histopathological features of a rhabdomyoma. A literature review revealed four reports of intracranial nerve rhabdomyoma, all of adult type and including one manifestation at the vestibular nerve. We present the first case of a fetal type extracardiac rhabdomyoma manifesting at a cranial nerve. Although rare, rhabdomyomas must be considered in the differential diagnosis of vestibular schwannomas.