Autoimmune hemolytic anemia (AIHA), which is caused by autoantibodies for red blood cell membrane antigens, is categorized into two forms: warm AIHA, which involves warm antibodies, and cold agglutinin disease (CAD), which involves hemolysis and red blood cell agglutination due to cold agglutinins. The first-line therapy for wAIHA is corticosteroids. Clinical guidelines by the British Society for Haematology recommend rituximab as second-line therapy, but Japanese national health insurance does not cover rituximab for wAIHA. Several new drugs with different mechanisms of action are in clinical development for refractory cases. Some of these drugs inhibit antibody production or promote antibody clearance, while others inhibit erythrophagocytosis. In CAD, anti-complement drugs targeting C1s improve anemia but do not treat peripheral circulatory failure due to erythrocyte aggregation. B-cell-targeted therapies should be used for patients with severe symptoms of these conditions.