Abstract
Ureteric leiomyosarcoma is a rare but aggressive tumor among other sarcomas. There is no established epidemiological data due to the scarcity of literature on this uncommon disorder. The present literature comprises about 20 case reports mostly of women above 40 years of age. The presenting complaint is mostly pain in the abdomen with only a few reporting urological symptoms like hematuria. Understandably, this tumor is diagnosed by histopathological examination with immunohistochemistry. We report one such case of a 32-year-old female who underwent an exploratory laparotomy with preoperative suspicion of adnexal neoplastic mass only to find normal ovaries and left ureteric tumor intraoperatively. She was managed with excision of the tumor with partial resection of the involved ureter and end-to-end anastomosis of the ureter followed by chemotherapy and radiation.