Patients exhibit benign tumors of the central and peripheral nervous system (e.g., schwannomas, meningiomas, ependymomas) and other non-tumorous findings such as ocular abnormalities (e.g., cataract, retinal hamartoma, epiretinal membranes), cutaneous manifestations (e.g., skin tumors, Fig. 1) [5, 6], or muscle atrophy due to NF2-associated neuropathy (Fig. 2) [6–8]. This evidence concerns the gene NF2 and schwannoma.