Cold agglutinin disease (CAD) is a rare and poorly understood disorder affecting 15% of patients with autoimmune hemolytic anemia (AIHA). In this case report, we describe a case of CAD mistaken for liver cirrhosis. A 57-year-old male patient was presumptive diagnosed with early liver cirrhosis due to mild hyperbilirubinemia and abdominal sonography finding without other findings as thrombocytopenia or prothrombin time prolongation. After 3 month, the patient was referred to our hospital with agglutination of peripheral red blood cell at room temperature and livedo reticularis of the whole body and finally diagnosed with CAD associated B cell lymphoproliferative disorder.