The most common of these is germline mutation of NF2 resulting in what was historically referred to as syndromic Neurofibromatosis type 2, an autosomal dominant condition characterized by the growth of multiple schwannomas and meningiomas.65 Due to the overlapping phenotypes of Neurofibromatosis type 2 and schwannomatosis, the latter being a tumor predisposition syndrome also characterized by the development of multiple schwannomas, the diagnostic criteria and disease nomenclature for NF2 and schwannomatosis was updated in 2022. Here, NF2 is linked to NF2-related schwannomatosis.