The past decade has seen tremendous advances in our understanding of the molecular and genetic basis of many neuropsychiatric disorders. Although the genetic aberrations that lead to these syndromes have been identified in many cases, not much is known about specific gene products and their function. This article reviews the molecular basis of well-known neurogenetic disorders. The syndromes discussed here follow a Mendelian pattern of inheritance and are predominantly single-gene disorders; however, most childhood and adolescent psychiatric disorders are polygenic in nature. This genetic complexity and heterogeneity has made it difficult to identify the genes involved in their etiology. Identification of genetic and environmental risk factors involved in the etiology of complex disorders, such as autism, will help in the discovery of medications that can ameliorate the symptoms.