Red blood cell (RBC) autoantibodies are a relatively uncommon cause of anemia. Nevertheless, autoimmune hemolytic anemia (AIHA) should consider in the differential diagnosis of hemolytic anemia whenever patient has an accompanying lymphoproliferative disorder, autoimmune disease, viral or mycoplasma infection. AIHA is further categorized as warm AIHA, cold agglutinin syndrome, paroxysmal cold hemoglobinuria, mixed-type and drug-induced AIHA. It is the characteristic of the autoantibodies which are accountable for various clinical entities. Diagnosis is based on clinical presentation and work up of AIHA. This review discusses epidemiology, causes, clinical presentation, and laboratory workup and treatment options available for AIHA.