A 33-year-old woman had a diagnosis of idiopathic Budd-Chiari syndrome complicated by inferior vena caval occlusion. Conservative medical therapy failed to control the symptoms of both portal hypertension and vena caval stasis. Therefore, a prosthetic shunt was placed from the right common iliac vein to the right atrium with a side-arm to the superior mesenteric vein. She exhibited almost complete relief of symptoms and the graft was documented to be patent two weeks postoperatively. In many instances aggressive surgical therapy may help these patients who, in the past, would have been relegated to symptomatic therapy.