Objective: The purpose of this study was to investigate the administration of bezafibrate alone and in combination with ursodeoxycholic acid (UDCA) in the treatment of primary biliary cirrhosis (PBC), Background: To the authors' knowledge, there has been no previous investigation of the effect of bezafibrate in the treatment of PBC, Methods: Of 30 patients diagnosed with PBC by means of a liver biopsy, 12 patients received bezafibrate 400 mg/d and 12 patients who had taken UDCA 600 mg/d for greater than or equal to 1 year, received bezafibrate 400 mg/d in addition to UDCA 600 mg/d, Drug administration continued for 12 months. The levels of alanine aminotransferase (ALT), alkaline phosphatase (ALP), gamma-glutamyl transferase (GGT), and immunoglobulin (IgM) recorded at 1, 3, 6, and 12 months were compared with pretreatment levels and the degree of change expressed as H%. Results: Laboratory test results decreased significantly during the study compared with pretreatment levels in both groups. No significant difference was noted between the 2 groups in terms of ALT, ALP, and IgM, although GGT decreased significantly in the bezafibrate-only group at 3, 6, and 12 months compared with the group receiving bezafibrate plus UDCA, Conclusion: Results of the present study suggest that bezafibrate, even when administered alone, may be potentially effective in the treatment of PBC.