Appendiceal endometriosis is an entity of extragonadal endometriosis. It commonly affects women of childbearing age with a wide spectrum of clinical manifestations. Women can present with symptoms mimicking acute appendicitis. Ultrasound, computed tomography scan (CT-Scan) and laparoscopy can be used for evaluation although the definitive diagnosis is obtained only with the histopathological study. METHODS We report a case of appendiceal endometriosis presenting as acute right lower quadrant abdominal pain in a woman of childbearing age. We discuss appendiceal endometriosis as a rare differential diagnosis of acute appendicitis in young women and the diagnostic challenges this condition may pose to general surgeons. CONCLUSIONS Appendiceal endometriosis is a rare condition with a challenging diagnosis. Although appendectomy relieves the acute symptoms, gynecological follow up is strongly recommended.