One such syndrome, neurofibromatosis type 2 (NF2) is an autosomal dominant inherited cancer predisposition syndrome, caused by a germline mutation in the NF2 tumor suppressor gene: 35–53% of individuals with NF2 develop ependymoma [7], with a striking predilection for the cervical or thoracic spinal cord (62–86% of tumors) [8]. This evidence concerns the gene NF2 and ependymoma.