We report herein the case of a 69-year-old woman in whom hepatocellular carcinoma (HCC) arising in the precirrhotic phase of primary biliary cirrhosis (PBC) was successfully managed by a right hepatic lobectomy. The patient, who had never received a blood transfusion, had a 4-year history of asymptomatic PBC of Scheuer's histological classification stage II. Abdominal computed tomography (CT) revealed a mass measuring 4.0 cm in the right hepatic lobe, and a right hepatic lobectomy was performed in consideration of her good liver function and the deep location of the tumor in the right lobe. The patient has remained well without any evidence of recurrent disease for 4 years since her operation. A review of the literature revealed only two cases of successful partial hepatectomy, but none of major hepatectomy. Most of the reported cases had been treated with transcatheter arterial embolization (TAE) and were associated with poor survival. Our experience of this patient indicates the potential value of hepatectomy as an alternative to TAE in selected patients with resectable disease and good hepatic function.