This article reviews extrapelvic endometriosis, emphasizing classic papers as well as recent research. Because of the nature of the existing literature, specifically case reports and retrospective analyses, this article is primarily descriptive in nature. Extrapelvic endometriosis is discussed based on some main areas of occurrence, including gastrointestinal, urinary, and thoracic; other areas are also reviewed. What is known about the epidemiology, pathogenesis, diagnosis, and treatment of extrapelvic endometriosis is highlighted. Areas for future direction of research in the field are also identified.