Hemolytic anemia can be caused by various hereditary or acquired diseases. Classification is usually based on corpuscular or extracorpuscular defects. Beside the anemia, laboratory testing indicates increased lactate dehydrogenase, unconjugated bilirubin and reticulocytes as well as reduced or absent plasma haptoglobin. Knowledge of further diagnostic procedures (e.g., Coombs test, schistocytes, hemoglobin electrophoresis or flow cytometric analysis) leads in many cases to an underlying disease with differentiated therapeutic options. Autoimmune hemolytic anemia (AIHA) is often associated with diseases as HIV, connective tissue disease, lymphomas or malignant tumors and the hemolytic process is preexisting in many cases. Thrombotic microvascular diseases (e.g., thrombotic thrombocytopenic purpura or hemolytic-uremic syndrome) are further important causes of hemolytic anemia which need immediate diagnosis and treatment.