Neurofibromatosis type 2 is a rare autosomal dominant phakomatosis manifesting as development of multiple CNS tumors. Unlike neurofibromatosis type 1 (NF1), it is not associated with neurofibromas.  Instead, patients with this disease have:

• intracranial schwannoma(s): mostly vestibular schwannoma(s)
• intracranial and spinal meningioma(s)
• intraspinal-intramedullary ependymoma(s)