Endometrial stromal sarcoma are rare malignant tumors of the uterus and has been described as the second most common malignant uterine mesenchymal tumor. The diagnosis is confirmed on hysterectomy for a presumed benign disease. In the latest World Health Organization (WHO) classification (2003), the term endometrial stromal tumor is applied to neoplasms typically composed of cells that morphologically resemble endometrial stromal cells of the nonneoplastic proliferative phase endometrium. The WHO recognizes three categories of endometrial stromal tumors: Endometrial stromal nodule, low-grade endometrial stromal sarcomas (LGESS), and undifferentiated endometrial sarcoma. We report here an interesting case of a 39-year-old female who presented with irregular bleeding per vaginum and urinary retention with a clinical impression of a leiomyomatous polyp, which on histological examination showed a LGESS.