Recently, the umbrella term “schwannomatosis” has been proposed to encompass the spectrum of syndromes characterized by the development of (or the predisposition to develop) schwannomas, [as opposed to the spectrum of syndromes characterised by the development of (or predisposition to develop) neurofibromas: i.e., NF1 and related disorders], including NF2 (now called NF2-related-schwannomatosis or NF2/MERLIN schwannoma predisposing syndrome) and schwannomatosis (now called SMARCB1-related and LZTR1-related-schwannomatoses or SMARCB1/LZTR1 schwannoma predisposing syndromes) (Table 7) [3]. This evidence concerns the gene NF2 and plexiform neurofibroma.