NF1 and NF2 are autosomal dominant hereditary disorders with germline mutations in NF1 and NF2 and clinically characterized by multiple benign nerve sheath tumors (especially neurofibromas in NF1, schwannomas in NF2), but also by a markedly increased risk on particular gliomas (especially pilocytic astrocytoma in NF1 and ependymomas in NF2) [33, 76, 184]. The gene discussed is NF2; the disease is pilocytic astrocytoma.