Although Hashimoto’s thyroiditis (HT) is commonly characterized by extrahepatic manifestations of primary biliary cirrhosis (PBC), coexistence of PBC and Graves’ disease (GD) is uncommon. This is a review of the English and Japanese scientific literature, comprising 7 cases of PBC and GD coexistence. All patients were female. In 4 cases, both diseases were almost simultaneously diagnosed, whereas PBC preceded GD in the remaining 3 cases. One fatality was observed on account of sepsis and liver failure caused by progressing PBC.