Autoimmune hemolytic anemia (AIHA) is a heterogeneous group of diseases caused by the attack of endogenous antibodies (autoantibodies) on erythrocyte antigens. It is divided into primary AIHA, which results from an immune response, and secondary AIHA, which is caused by disorders of immune regulation. The incidence of AIHA is 1 to 3 cases per 100,000 people per year, and it is diagnosed by direct (DAT) and indirect (IAT) antiglobulin tests. Based on the diagnostic tests and the characteristics of the autoantibodies, AIHA is divided into warm (wAIHA), cold (cAIHA), and mixed forms. In addition, cold AIHA is subdivided into cold agglutinin disease (CAD) and cold agglutinin syndrome (CAS), depending on the etiopathogenesis. Paroxysmal cold hemoglobinuria (PCH) is a rare form of autoimmune hemolytic anemia in which autoantibodies bind to erythrocytes at low temperatures and activate the complement system at high temperatures. With activation of the complement system and formation of the membrane attack complex (MAC), hemolysis may be caused by activation of the ADCC mechanism or phagocytosis of erythrocytes by macrophages. Disorders of genes regulating the immune system, infection, environment, drugs, and tumor formation are possible causes of AIHA. Considering the heterogeneity of the disease and the different immunopathogenic mechanisms of AIHA, therapies may differ widely. The first line of treatment is glucocorticosteroids, followed by rituximab, but also splenectomy. Immunosuppressive drugs, cyclosporine, azathioprine, and cyclophosphamide, make up the third line of therapy. Clinical trials of new therapies include erythrocyte phagocytosis inhibition (fostamatinib, nipocalimab), Bruton tyrosine kinase inhibition (rilzabrutinib and ibrutinib), proteasome inhibition (bortezomib), monoclonal antibodies to CD38 (daratumumab, isatuximab), mTOR inhibition (sirolimus), and complement protein inhibition (eculizumab, pegcetacoplan, sutimlimab).