Abstract:  Neurofibromatosis type II (NF-2) is a syndrome characterized by the development of multiple central nervous system neoplasms, most commonly acoustic nerve schwannomas, meningiomas, and spinal tumors. It is considered to be one of the phakomatoses and, until 1980, it was not recognized as an entity distinct from von Recklinghausen's neurfibromatosis (NF-1).