Objective To explore the relationship of therapeutic compliance and complications in children with severeβ-thalassemia.Method One hundred and eight children with severeβ-thalassemia involved in the survey using convenient sampling to investigate the relationships between compliances with blood transfusion and iron removal treatment and complications.Results Of all the patients,66 complied with blood transfusion and the rest 42 did not;73 complied with the therapy and the rest 35 did not. Those complying with the blood transfusion had significant lower rates of developmental retardation,unusual faces,hepatosplenomegaly(P<0.01). Those complying with the therapy had significant lower rates of injuries in liver,heart and endocrinal system(P<0.01).Conclusion Therapeutic compliance in children with severeβ-thalassemia is at lower level so that the incidence of complications in them is higher.