A 64-year-old male, who had been treated for liver dysfunction and esophageal varices without infection of hepatitis B or C for the previous 10 years, was referred to our department for treatment of a round mosaic lesion, 6cm in diameter, in the medial segment of the liver. On admission, imaging diagnoses revealed a complete occlusion of the infrahepatic vena cava, 3cm in length, and abundant collateral circulations. Angiography revealed a hypervascular tumor in the medial segment of the liver and an occlusion of the infrahepatic poriton of the inferior vena cava just above the short hepatic vein. Serum AFP was 1176ng/ml and PIVKA-II was 0.6AU/ml. The patient was diagnosed as hepatocellular carcinoma combined with Budd-Chiari syndrome. After intrahepatic arterial infusion therapy, when the tumor diameter decreased to 3.5cm and the combined liver cirrosis was in clinical stage II, medial segmentectomy was carried out. Macroscopically the resected tumor was multinodular fusion type. Histological examination of the resected specimen showed moderately differentiated hepatocellular carcinoma (Edmondson II-III) and congestive liver cirrhosis with proliferation of small vessels in the portal space-septa.