Endometrial stromal sarcoma (ESS) rarely infiltrates the great vessels. We report a successful surgical resection of the inferior vena cava (IVC) after extensive infiltration with metastatic low-grade ESS. A case of presumed recurrence of low-grade ESS demonstrated complete IVC occlusion from tumour thrombus with extensive local disease. Radical resection of the tumour and caval reconstruction was performed. The IVC graft was thrombosed at short-term follow-up. Curative resection of extensive caval infiltration with metastatic low-grade ESS can be achieved. Caval reconstructive procedures may be redundant in the presence of an adequate collateral circulation.
