We report herein the case of a patient with primary biliary cirrhosis (PBC) with hepatocellular carcinoma (HCC) who underwent anatomical hepatectomy. A 74-year-old man with PBC was found to have a 3.4-cm liver tumor close to the right hepatic vein in segment 7 on follow-up CT study. HCC was diagnosed and extended right posterior sectionectomy involving the right hepatic vein was performed. The technical difficulty of transection of the hepatic parenchyma with PBC seemed comparable to that in cases of other causes of liver cirrhosis, and no particular perioperative complications were encountered. Histological study revealed moderately differentiated HCC and Scheuer grade IV PBC. Even in the case of PBC, hepatectomy can be safely performed according to the indications on the basis of cancer progression and hepatic functional reserve, as in other etiologies of liver disease.