We report two cases of nodular regenerative hyperplasia of the liver associated with primary biliary cirrhosis. Cholestasis and presence of antimitochondrial antibodies were noted in both patients. In one patient, the diagnosis of nodular regenerative hyperplasia was supported by the demonstration of disseminated small hepatic nodules without perinodular fibrosis. Twelve years later, the histopathological picture was one of primary biliary cirrhosis. The other patient presented an histological picture of regenerative hyperplasia of the liver and primary biliary cirrhosis. The association of regenerative hyperplasia of the liver and primary biliary cirrhosis is discussed.