Benign metastatic leiomyomatosis (BML) is a rare disease characterized by smooth muscle cell proliferation in extrauterine sites including the lung, abdomen, pelvis, and retroperitoneum. Depending on location, BML is classified as intravenous leiomyomatosis and diffuse peritoneal leiomyomatosis. Pathogenesis of BML can be iatrogenic after previous myomectomy or hysterectomy, hormonal, or coelomic metaplasia. Treatment options are observation, hormonal suppression, and/or surgical debulking via laparotomy or laparoscopy. Laparoscopic surgery is gaining in popularity in the gynecologic field compared to laparotomic surgery and single-port laparoscopy has the benefits of cosmesis and early tissue extraction by transumbilical morcellation. We report a 39-year-old woman with BML who underwent single-port laparoscopy debulking surgery.