Twenty-six patients with the clinical and histologic diagnosis of primary biliary cirrhosis were reviewed. Nodular hyperplasia of the liver without fibrous rims, not reported hitherto in patients with primary biliary cirrhosis, was found in several patients with early histological stages. These changes resembled “nodular regenerative hyperplasia of the liver” and were usually present as multicellular thickness in zones 1 and 2 of the hepatic lobules. Such lesions were preferentially found in patients with esophageal varices, suggesting that the nodular hyperplasia may occur in relation to a disturbance of portal venous blood flow within the liver in primary biliary cirrhosis in early histological stages.