Leiomyosarcoma of the Inferior Vena Cava (IVC) is a rare soft tissue tumour which accounts for only 0.5% of all soft tissue sarcomas. The patients usually presented with non specific clinical signs and often diagnosed at advanced stage. We reported a case of a 58-year-old lady who presented with right sided abdominal pain for 6 months. CT scan showed large Level 2 IVC tumour which encased the right renal vein and had no clear plane of demarcation with the caudate lobe of the liver. She underwent radical resection of the IVC tumour with the right kidney removed en bloc. Vascular reconstruction was not performed as established collateral veins present. She was nursed in Intensive Care Unit for 2 days and discharged well at day 5 post operatively. Histopathological examination results revealed leiomyosarcoma of IVC.
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