Neurofibromatosis type 2 is an extremely rare form of neurofibromatosis characterized by central nervous system involvement with bilateral vestibular schwannomas and spinal tumors. Anesthetic management of a parturient with neurofibromatosis type 2 has not been fully reported, and the condition is challenging to obstetric anesthesiologists due to the presence of intracranial and intraspinal canal neurofibromas. We present a case of neurofibromatosis type 2 referred for delivery. Because of central neuraxial involvement, regional anesthesia was avoided, and the patient delivered by cesarean section under general anesthesia. The importance of pre-operative diagnosis and multidisciplinary management for neurofibromatosis type 2 is emphasized and anesthetic and obstetric considerations for delivery are presented.