Patients with primary biliary cirrhosis (PBC) are at increased risk for various malignancies including lymphoproliferative diseases. In this report, we describe a 52-year-old man with the simultaneous diagnosis of PBC and IgG-kappa multiple myeloma (MM). Serum cholestatic enzyme (alkaline phosphatase and gamma-glutamyltransferase) levels decreased after three courses of vincristine, adriamycin and dexamethasone treatment followed by six courses of melphalan and methylprednisolone, given for MM. Pruritus also disappeared. He did not show any progression during the next 24 months of clinical follow-up. The coexistence of PBC and MM is rare and the pathogenetic mechanism under this association remains unclear. Clinical improvement of both diseases in this patient after chemotherapy may indicate an association; however, absence of concurrency in previous large series increases the possibility of coincidence in this case. When compared with the previous single case reports, this case provided long-term results about response after chemotherapy.