The authors present a case of leiomyosarcoma of inferior vena cava presenting as Budd-Chiari syndrome. A thirty-two-year-old man presented with abdominal distension, edema feet and cough. Chest x-ray showed a non-homogenous opacity in the right upper zone of lung with adjacent rib destruction. CT scan of abdomen revealed a nodular mass inside the inferior vena cava at the level of hepatic vein and venogram showed complete obstruction at that level. Needle biopsy specimen from the lesion was suggestive of leiomyosarcoma.