Autoimmune hemolytic anemia (AIHA) encompasses a group of disorders characterized by a shared pathophysiological mechanism of immune-mediated destruction of self-red blood cells, with notable variations in etiology, clinical manifestations, and, consequently, treatment strategies. Warm and cold AIHA account for most cases of the disease. In warm autoimmune hemolytic anemia (wAIHA), hemolysis primarily occurs through immunoglobulin G (IgG)-mediated extravascular destruction of red blood cells, whereas cold autoimmune hemolytic anemia (cAIHA) is distinguished by intravascular hemolysis, driven by Immunoglobulin M (IgM) antibodies and complement activation. Comprehensive evaluation of all patients is essential to identify underlying primary triggers, including infections, connective tissue diseases, lymphoproliferative disorders, myelodysplasia, and immunodeficiency. The direct antiglobulin test (DAT), along with its subspecificity and cold agglutinin titer, serves as a key diagnostic tool for characterizing various subtypes of AIHA. In cases of true DAT-negative AIHA, specialized laboratory investigations employing advanced technologies are required for accurate diagnosis. Once AIHA is confirmed, treatment is generally well-established, with steroid with or without steroid-sparing agents being the mainstay for wAIHA, while cold agglutinin disease (CAD) is managed through complement inhibitors and therapies targeting plasma cells. Supportive therapies, including temperature regulation for CAD, folic acid supplementation, thromboprophylaxis, and measures for osteoprotection, play a critical role in patient management and should be given due consideration. However, a subset of patients may exhibit refractory disease despite standard treatment approaches, necessitating consideration of experimental therapies.