Cold Agglutinin Disease (CAD) is a rare type of autoimmune hemolytic anemia (AIHA) characterized by the unique property of IgM autoantibodies binding to red blood cells (RBCs) at low temperatures. Agglutination of RBCs and activation of complement are precursors to expression of clinical features manifested as ischemic changes and hemolysis. Avoidance of cold exposure is necessary for all patients. Some patients require immunosuppressive agents, anti-CD20 therapy, plasma exchange and, if present, treatment of underlying disease. Overall prognosis in primary CAD is favorable but variable in the presence of B-cell neoplasms. This case report is a reminder of Cold Agglutinin Disease and the importance of recognizing it in clinical practice.