BACKGROUND Low-grade endometrial stromal sarcoma is a rare neoplastic growth in the uterine cavity, representing less than 1% of uterine tumors. Such tumors usually affect premenopausal and perimenopausal women, with a mean age of 46 years. Treatment generally starts with surgical resection of the tumor, followed by chemotherapy, radiotherapy, or hormonal therapy. METHODS In the current report, we again present a case of low-grade endometrial stromal sarcoma in a 51-year-old Mediterranean woman presenting with abdominopelvic pain. Computed tomography scan revealed a primary uterine tumor measuring 17 × 9 × 9 cm metastasizing to the lungs, bladder, and ureteral orifice, along with lymphovascular involvement. The patient underwent total abdominal hysterectomy, omentectomy, and lymph node dissection. Estrogen deprivation was accomplished by bilateral salpingo-oophorectomy. Lifelong hormonal therapy consisting of letrozole 2.5 mg per day was prescribed, which demonstrated remarkable efficacy, resulting in a partial remission of lung metastasis within 8 months after surgery. Full remission was observed after 18 months of hormonal therapy, with no recurrence. Another scan was performed after 2.5 years, revealing complete remission with no recurrence. CONCLUSIONS We again report a case of complete remission of low-grade endometrial stromal sarcoma after surgical removal of the tumor along with first-line hormonal therapy without the use of chemotherapy or radiotherapy, emphasizing the role of hormonal therapy in the treatment of such tumors.