The acoustic neuroma, which presents the symptoms of gradually progressing unilateral sensorineural hearing loss, tinnitus and vertigo, generally arises from the vestibular schwann sheath cells within the internal auditory canal. The symptoms of sudden deafness and facial paralysis are unexpected presenting complaints in patients with acoustic neuroma. Recently we experienced a 24 year-old male patient who complained of sudden deafness and rapid-onset facial paralysis of the ipsilateral side, and recested completely the tumor through translabyrinthine and middle fossa approach. The acustic neuroma can present atypically with sudden deafness and rapid-onset facial paralysis, so we report its clinical manifestations and characteristics with review of literatures.