Glucose-6-phosphate dehydrogenase deficiency (G6PD) is one of hereditary diseases sariously influencing the human health. G6PD is characterized by wide distribution, high incidence, inducing the hemolysis, complex mechanism of hemolysis and common occurence in children and so on. The blood transfusion is most effective method for acute ouset of hemolysis, but the risk is more high, thereby it is necessary to guarante the safety of blood transfusion. In addition of the routine managementin blood transfusion and standard procedures, the basic rules and indicators of blood transfusion must be grasped, the blood preparations should be known well, the blood protection must be strengthened, and the measures of personalized psychologic interference must be drawn up carnestly and performed strictily so as to improve the therapeutic efficacy and safe of blood transfusion. Through the promotion and development of social pubbicity, elucation activities, preventive and control measures and medical levels, many patients have been freed from healthy problems as soon as possible. In this review, the research progress on acute hemolysis and safe blood transfusion in G6PD are summarized.