A 48-year-old woman with primary biliary cirrhosis (PBC) and multiple sclerosis (MS) is reported. She presented with a visual deficit in 1975. In the course of a diagnostic evaluation, she was found to have PBC. In 1986, the diagnosis of MS was made. The patient's liver function deteriorated and liver transplantation was performed in May 1988. This is the third known case of this association.