Mutations in the SMARCB1 (otherwise known as INI1, BAF47, or hSNF5 gene and centromeric to the NF2 gene situated on chromosome 22) and LZTR1 genes (also located on chromosome 22, close to the former gene), which suppress tumors, are associated with this type of neurofibromatosis [16,18]. Here, NF2 is linked to neurofibromatosis.