Extrapelvic endometriosis is a rare condition defined as the presence of endometriotic stroma and glands outside the pelvis and elsewhere in the body. The cervix, vagina, vulva, intestinal tract, urinary tract, diaphragm, abdominal wall, inguinal canal, thoracic cage and lungs, extremities and even the central and peripheral nervous system can be involved. Because extrapelvic endometriosis is located in unusual sites, it is often confused with other pathologic conditions. This can lead to a difficult and challenging diagnosis and management. In the presence of recurrent, cyclical and catamenial symptoms, extragenital endometriotic lesions should be suspected. The aim of our paper is to report 9 cases of rare locations of extrapelvic endometriosis and to provide a literature review. © 2014 Wichtig Publishing.