Endometrial stromal sarcoma (ESS) represents only 0.2％ of all uterine malignancies. Even fewer cases have been reported on patients presenting with symptomatic disease. We report the case of young lady presenting with a large bleeding abdominal mass. At the time of surgery, an en bloc excision of the mass with a partial pancreatectomy, splenectomy, transverse colectomy, left nephrectomy, left adrenalectomy and resection of the diaphragm was performed. The final pathology revealed a malignant spindle and epithelial cell neoplasm with features favoring a variant of ESS. ESS can present in an atypical fashion as an intra-abdominal mass; a multi-disciplinary treatment approach for patients with this disease is crucial.