A review of the published literature found 60 congenital and acquired disorders with symptoms that include psychosis in youth. The prevalence, workup, genetics, and associated neuropsychiatric features of each disorder are described. Eighteen disorders (30%) have distinct phenotypes (doorway diagnoses); 18 disorders (30%) are associated with intellectual disability; and 43 disorders (72%) have prominent neurologic signs. Thirty-one disorders (52%) can present without such distinct characteristics, and are thus more easily overlooked. A systematic and cost-effective differential diagnostic approach based on estimated prevalence and most prominent associated signs is recommended.