Nervous system tumors represent unique neoplasms that arise within the central and peripheral nervous sys-tem.While the vast majority of nervous system neoplasm occur sporadically,most of the adult and pediatric formshave a hereditary equivalent.In a little over a decade,we have seen a tremendous increase in knowledge of theprimary genetic basis of many of the familial cancer syndromes that involve the nervous system,syndromes that aremostly inherited as autosomal dominant traits.In this review,we discuss the most recent findings on the geneticbasis of hereditary nervous system tumors.The identification of genes associiated with familial cancer syndromes hasin some families enabled a“molecular diagnosis”that complements clinical assessment and allows directed cancersurveillance for those individuals determined to be at risk for disease. Nervous system tumors represent unique neoplasms that arise within the central and peripheral nervous sys-tem. Whilst the vast majority of nervous system neoplasm occur sporadically, most of the adult and pediatric formshave a hereditary equivalent. In a little over a decade, we have seen a tremendous increase in knowledge of the primary genetic basis of many of the familial cancer syndromes that involve the nervous system, syndromes that are mostly inherited as autosomal dominant traits. in this review, we discuss the most recent findings on the genetic basis of hereditary nervous system tumors. The identification of genes associiated with familial cancer syndromes has some of the families enabled a “molecular diagnosis ” that complements clinical assessment and allows directed risks of cancer for those who determined to be at risk for disease.