Although autoimmune diseases are often concomitant, the coexistence of primary biliary cirrhosis (PBC) and autoimmune hemolytic anemia (AIHA) is not common. This is a review of the English and Japanese literature, regarding concomitant cases of PBC and AIHA. Among the 23 concomitant cases, PBC was diagnosed first in 10 cases and both diseases were almost simultaneously diagnosed in the remaining 13 cases. This suggests that there have been few concomitant cases in which AIHA developed first before the development of PBC. Moreover, there may be no correlation between the occurrence of AIHA and the staging or progression of PBC.