Current clinical criteria identify patients with definite schwannomatosis when they are more than 30-years old and have all of the following features: two or more non-intradermal schwannomas with at least one histologically proven, no vestibular schwannomas, and does not meet NF2 diagnostic criteria nor have a first- degree relative with NF2 nor have a known NF2 variant [5]. This evidence concerns the gene NF2 and schwannomatosis.