Smooth muscle sarcoma (LMS) is a malignant tumor composed of smooth muscle cells or cells that differentiate into smooth muscle cells. Sarcomas originating from the urogenital system are rare. Among adults, smooth muscle sarcomas are more common, while rhabdomyosarcoma is most common in children. To date, there are reports of smooth muscle sarcomas occurring in the ureter, bladder, and urethra. Bladder smooth muscle sarcomas are reported most frequently, followed by those occurring in the urethra. The source may be urothelial smooth muscle tissue or vascular smooth muscle tissue.