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Endometrial stromal sarcoma (ESS) is a mesenchymal neoplasm that usually occurs as a primary tumor of the uterine corpus, but rarely arises in other sites, such as the ovary, pelvic cavity, mesentery, omentum and intestine. Endometrial stromal sarcoma is a malignant subtype of endometrial stromal tumor arising from the stroma (connective tissue) of the endometrium rather than the glands. There are three grades for endometrial stromal tumors. All the cases yet reported in literature had a history of previous hysterectomy with or without a diagnosis of ESS. Here we present a unique case of Low grade ESS where the patient presented with vague abdominal pain, loss of weight and anemia. On evaluation with USG and CT demonstrated a lobulated mass in mesentery which led to the diagnosis of a GIST from small bowel. Intraoperatively the lesion was in omentum with no attachment to mesentery. The enlarged uterus with fibroids were also removed along with the omental mass after a clinicopathological discussion which suggested the possibility of ESS with primary in the uterus. The histopathology demonstrated Low grade Endometrial sarcoma which was supported by immunohistochemistry

GIST or Not - A Unique Case of Low Grade Endometrial Stromal Sarcoma with Review of Literature