Autoimmune haemolytic anaemia (AIHA) is a rare disease. In clinical practice, diagnosis and treatment of AIHA turns out to be troublesome. Correct diagnosis is dependent on proper comprehension of the pathophysiology and the laboratory tests performed by the transfusion laboratory. The present review provides a short overview on the pathogenesis of autoimmune haemolytic anaemia. The diagnostic pitfalls will be discussed and a diagnostic algorithm for proper diagnosis of AI HA will be given. Moreover, a brief overview on the treatment of different forms of AIHA is given.