A 29 year old woman with unresectable leiomyosarcoma of the inferior venal cava extending into the right atrium of the heart was presented. The upper and middle inferior vena cava was involved and there were both intrinsic and extrinsic and extrinsic tumor growth components. The upper and middle inferior vena cava was involved and there were both intrinsic and extrinsic tumor growth components. The diagnosis was made by a combination of radiological studies including ultrasound, CT, inferior vena cavography and arteriography and the diagnosis was confirmed by ultrasound guided tumor biopsy. The clinical and radiological presenting features were reviewed.