A 71-year-old female with primary biliary cirrhosis, multiple myeloma and hypothyroidism is reported. The liver biopsy showed stage II-III histologic damage according to Scheuer's classification and the multiple myeloma was IgG-lambda type, stage II-A at the time of diagnosis. Another three cases of primary biliary cirrhosis associated with multiple myeloma were found in the literature. As a group, the four cases had mild or moderate liver damage, extensive bone lesions and three of four cases had multiple myeloma IgG-lambda type. Although this association could be incidental, there is evidence that implies a pathogenic relationship. This information is summarized in this report.