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. The gene discussed is NF2; the disease is NF2-related schwannomatosis.