Endometrial stromal sarcomas are rare malignant tumours of the uterus. Most details about this tumour is obtained from previous case reports. In most of the cases, diagnosis is made only postoperatively. A 29 year old female, came with complaints of heavy menstrual bleeding, lower abdominal pain, mass in lower abdomen for 2 months. Clinical examination and investigations showed bulky irregularly enlarged uterus probably Fibroid Uterus. Patient was planned for Myomectomy. Intraoperatively, uterus was irregularly enlarged with cauliflower like growth of size 7 x 5 cm over fundus suggestive of Carcinoma. Total abdominal hysterectomy with bilateral salphingo oophorectomy with bilateral pelvic lymphnode dissection with infracolic omentectomy was done. Histopatholgy examination revealed as Low Grade Endometrial Stromal Sarcoma with Negative Lymph Node Spread. Postoperatively, Patient was evaluated for ER sensitivity and started on Tab. Anastrazole. © 2023 Elsevier B.V., All rights reserved.