Multiple neurofibromatosis is known as Von Recklinghausen's disease, and is an uncommon congenital, familial disease characterized by the presence of a typical skin manifestation consisting of multiple cutaneous tumors and ""cafe-au-lait"" spots. Neurofibromatosis affects primarily cell growth of neural tissues and can cause tumors to grow on nerves at any time and any location, and neurofibromatosis type II is characterized by bilateral acoustic neuromas with high risk of other tumors. The authors experienced a case of neurofibromatosis occuring in the left potic nerve, bilateral trigeminal nerves, both acoustic nerves, and cervical spinal cord, and removed the Lt, side acoustic neuroma successfully via translabyrinthine approach without significant complication.