Ninety percent of schwannomas are isolated, and 4% of schwannomas are associated with NF2, while the remaining 5% of schwannomas are varied but not related to NF2.[8] The incidence of NF2 is low, approximately 1/25000–40000, and NF2 is often accompanied by bilateral vestibular schwannomas.[9] NF2 is associated with more aggressive biological behavior of schwannomas, and all young patients with NF2 often develop multiple schwannomas with a higher risk of malignant transformation. This evidence concerns the gene NF2 and schwannoma.