NF2 (Neurofibromatosis type 2)-related schwannomatosis (NF2-SWN) is an autosomal dominant disorder whose characteristic features include multiple nervous system tumors (vestibular schwannomas, intracranial meningiomas, spinal ependymomas, and peripheral nerve tumors), ocular abnormalities, and skin lesions [10]. The gene discussed is NF2; the disease is peripheral nervous system neoplasm.