The familial occurrence of bilateral acoustic neuroma has rarely been reported in the literatures, and its inheritance is known to be an autosomal dominant trait in association with or without von Recklinghausen's disease. Recently, we experienced an unusual case of bilateral acoustic neuroma, which was familially occurred in mother and her son. A 18-year-old Korean boy was referred to us due to bilateral hearing disturbance and staggering gait of one year duration. There were no stigmata of von Recklinghausen's disease. Neurological examination, simple skull films and vertebral angiograms revealed various evidences of bilateral cerebellopontine angle tumors. At operation, a hen egg-sized firm mass was subtotally removed at the left cerebellopontine angle region and a peanut-sized mass was totally removed at the right cerebellopontine angle region via suboccipital craniectomy. The histological diagnosis was neurofibroma. In family history, 13 years ago his mother was operated on C6-7 neurofibroma at her age of 28 years, and again operated on bilateral acoustic tumors 2 years later. The histological diagnosis was also neurofibroma.