Abstract
Primary urachal leiomyosarcoma is rare entity with unclear prognosis and treatment strategy. We report a case of urachal leiomyosarcoma presenting, and treated with open urachal tumor resection and partial cystectomy. Pathological diagnosis showed low-grade leiomyosarcoma. At 53-months follow-up, no recurrence or metastasis was found. The purpose of this case is to raise awareness of the disease.