Introduction Sarcomas of vascular origin are rare. A case of Ewings sarcoma of Inferior Vena Cava [IVC] is reported here. Report A 36-year-old lady presented with a 6-month history of bilateral pitting lower limb swelling and 1-month history of vaginal bleeding. Contrast-enhanced computed tomography of the thorax and abdomen revealed a soft tissue lesion extending from the cavoatrial junction to the junction of the middle hepatic vein with IVC. She underwent IVC resection (suprahepatic) and left lateral hepatectomy with dacron graft reconstruction. The pathology was suggestive of Ewings sarcoma and she received adjuvant chemotherapy. Conclusion Preoperative tissue diagnosis can be considered in vascular tumors to sequence the various treatment modalities for improved outcomes.