Endometriosis, while prevalent, can manifest in extra-pelvic locations with varying degrees of rarity, but reports of multiple extra-pelvic sites within a patient are extremely rare. We report a unique case of a 45-year-old female with concurrent inguinal endometriosis and catamenial pneumothorax. The patient experienced recurrent menstruation-associated right chest pain and subsequently developed right inguinal pain. A laparoscopic-assisted en bloc resection of the round ligament and associated inguinal mass was performed. Intraoperative findings were consistent with pelvic endometriosis. Hormonal therapy was not initiated due to the patient desiring pregnancy, but despite assisted reproductive technology, pregnancy was unsuccessful. Subsequent investigation following the onset of right chest pain and dyspnea revealed right pneumothorax. Thoracoscopic intervention identified diaphragmatic defects suggestive of endometriosis. Resection of the lung parenchyma with an air leak, along with suture repair of a diaphragmatic defect, was performed. Postoperatively, the pneumothorax resolved. Post-surgical hormonal therapy with dienogest resulted in the resolution of both conditions. This case underscores the potential for diverse presentations of extra-pelvic endometriosis and highlights the importance of a multidisciplinary approach to its management.