The central nervous system can be affected in many hereditary tumor syndromes, also sometimes referred to as familiar tumor syndromes, genetic tumor syndromes or cancer predisposition syndromes. Although a few of these have been recognized for a long period of time and have been well delineated with regard to clinical presentations and genetic abnormalities, some have been only recently defined. These disorders are highly complex and patients are best cared for in specialized centers which have broad interdisciplinary expertise to handle the wide array of phenotypic manifestations of these disorders. This book contains a collection of chapters which focus on a subset of these syndromes. The focal of the chapters in this text is on the neuropathologic features associated with the various syndromes that are highlighted.