Budd-Chiari syndrome is a clinical disorder caused by hepatic venous obstruction with manifestations of abdominal pain, hepatomegaly, and ascites. Secondary Budd-Chiari syndrome is defined as an obstruction that results from material not originating from the venous system. We describe a rare case of fulminant Budd-Chiari syndrome secondary to renal cell carcinoma with tumor thrombus of the inferior vena cava and hepatic veins. The 59-year-old man was admitted to our hospital because of progressive appetite loss and markedly elevated serum transaminase. Abdominal ultrasonography revealed thrombosis in the hepatic veins and the inferior vena cava. Renal cell carcinoma with hepatic vein invasion was suggested by abdominal computed tomography and confirmed after a biopsy was taken from the hepatic venous thrombus. The patient died of fulminant liver failure within 10 days after admission. The clinical scenario and rationale for the selected management are further discussed.