(I) Overview NF2 often presents as neoplastic or dysplastic lesions of Schwann cell tumors (nerve sheath tumors or nerve sheath hypertrophy), meningeal cell lesions (meningiomas or meningeal hemangiomas) and glial cell lesions (gliomas and glial malformations), and others (lens clouding and cerebral calcifications). (b) Epidemiology The incidence of NF2 is about 1/5000. (c) Genetics NF2 is an autosomal dominant disorder with the gene located at 22q12. (d) Clinical manifestations NF2 manifests in 3 main forms ① Multiple intracranial nerve Schwann cell tumors occur in about 85% of the 8th pair of cranial nerves, especially bilateral vestibular branch Schwann cell tumors, followed by the 9th cranial or spinal nerve; most of them develop before the age of 30 years. (ii) 50% of the patients present with subcutaneous neurofibrillary tumors. (ii) 50% present with subcutaneous neurofibromas and another 25% with cutaneous manifestations (iii) 40% present with clouding of the lens and no iris malformations. (E) Treatment The disease is genetically inherited and has no cure. It can only be treated by surgical excision of the lesion causing the patient’s symptoms, and may require multiple surgical procedures during the patient’s lifetime.