NF2 and neoplasm: NF2-related Schwannomatosis (NF2-related SWN) previously known as Neurofibromatosis type 2 is an autosomal dominant, tumor predisposing condition arising due to a mutation in the NF2 tumor suppressor gene.1–3 The incidence at birth is approximately 1:25 000–33 000.4 One of the hallmarks of NF2-related SWN is the development of bilateral vestibular schwannomas (VS) involving the vestibular branch of the eighth cranial nerve with hearing loss as one of the main causes of morbidity.1,2