A case of Hurthle cell adenoma and Graves disease associated with primary biliary cirrhosis is reported in a 51-year-old woman, who developed elevated liver enzymes and hyperthyroidism. Thyroid disorders accompanying primary biliary cirrhosis, especially hypothyroidism, are well documented. Concomitance of primary biliary cirrhosis and hyperthyroidism is rare. Moreover, there has been no previous report of a case with Hurthle cell adenoma accompanied by Graves disease and primary biliary cirrhosis.