A 29-year-old Japanese female was diagnosed with systemic lupus erythematosus (SLE) at the age of 21 and went into remission after administration of prednisolone. Although no liver dysfunction had been observed upon diagnosis of SLE or during follow-up, an increase of hepatobiliary enzyme levels was seen eight years after the diagnosis of SLE. Antimitochondrial antibodies were positive. Cell infiltration around intrahepatic bile ducts and granuloma formation were observed in the liver. Therefore, she was diagnosed with primary biliary cirrhosis (PBC). Administration of ursodeoxycholic acid resulted in normalization of hepatobiliary enzyme levels. Development of PBC after SLE is extremely rare.