ABSTRACT : Both primary biliary cirrhosis (PBC) and autoimmune hemolytic anemia (AIHA) are uncommon diseases. Immunological dysregulation is suggested as a causative factor for both diseases. We report a 77-year-old woman who suffered from warm type AIHA complicated by PBC. Her direct antiglobulin test was positive for IgG, and negative for C3. Both anti-mitochondrial antibody and its M2 component were detected. Both alkaline phosphatase (Alp) and IgM were elevated in the serum. She was initially treated with steroids for 8 months. Her steroids were discontinued when she underwent a laparoscopic splenectomy. Ursodeoxycholic acid was discontinued due to an allergic skin reaction. Her Alp improved with bezafibrate.