Background: Endometriosis, defined as endometrial glands and stroma outside the endometrial cavity, affects up to 10% of reproductive age women. Implants outside of the pelvis, extrapelvic endometriosis, may occur in up to 15% of endometriosis patients. A patient with two rare manifestations of extrapelvic endometriosis; umbilical and thoracic including catamenial pneumothorax is presented. Case: A 43-year-old white woman had a history of intermittent umbilical bleeding beginning at age 23. She was diagnosed at age 42 with a right spontaneous pneumothorax occurring perimenstrually. Six months later, the patient had an umbilical 8-mm purple-bluish endometriotic papule excised. She has since experienced two additional menstrually related pneumothoraces. Conclusions: Histopathology of the umbilical lesion revealed endometriosis. Thoracoscopy at the time of the third pneumothorax showed multiple endometriotic lesions on the diaphragm, similar in appearance to pelvic lesions. Mechanical pleurodesis and partial apical pleurectomy were performed. The patient was started on gonadotropin-releasing hormone agonist, with a plan of hysterectomy and oophorectomy. A case is reported of a patient presenting with two rare manifestations of extrapelvic endometriosis: umbilical and thoracic. It allows a multispecialty review of endometriosis, the theories regarding the etiology, and treatments (including minimally invasive surgery) available for patients with extrapelvic endometriosis. ( J GYNECOL SURG 27:95)