Familial melanoma-astrocytoma syndrome is now appreciated to represent an autosomal-dominant variant of the familial melanoma syndrome caused by heterozygous germline CDKN2A inactivation that also includes development of astrocytomas and occasionally other neural tumors including peripheral nerve sheath tumors and meningioma (Online Mendelian Inheritance in Man, entry # 155755). Here, CDKN2A is linked to meningioma.