Schwannomatosis is a rare form of neurofibromatosis that has only recently been defined.  Similar to people with neurofibromatosis type 2 (NF2), people with schwannomatosis develop multiple schwannomas on cranial, spinal and peripheral nerves.  However, unlike NF2, they do not develop vestibular schwannomas. Additionally, they do not typically develop other types of tumors associated with neurofibromatosis such as meningiomas, ependymomas, neurofibromas or astrocytomas and there does not seem to be an increased risk of developing malignancies.  People with schwannomatosis often have problems with pain.  In many patients this is their only symptom.

Like NF1 and NF2, not everyone with schwannomatosis will have the same symptoms, and the severity can vary between patients.