We herein report on the current status of Japanese HIV-positive patients with coagulation disorders, primarily hemophilia, based on the national survey of 31 May 2006. The total number of registered patients was 1,431 (Hemophilia A 1,086; Hemophilia B 325; von Willebrand disease 8; others 12), and 604 of these patients were deceased by 31 May 2006. The survival rate after the beginning of 1983 was evaluated by the Kaplan-Meier method. The total number of surviving patients was 827, and the survival rate on 31 May 2006 was 55.7 +/- 1.4%. Among the 827 surviving patients, HCV antibody was observed in 740, was negative in 16, and was not reported in 71 patients. Thus, the prevalence of HCV infection was 98% in the surviving patients based on the presence of HCV antibody. Among the 604 deceased patients, liver disease was reported as a cause of death in 149 cases (25%), and infection with HCV was reported as the possible cause of liver disease in 120 cases (20%). After 1997, 63 cases among the subtotal of 148 deaths had critical hepatic disease that originated from HCV infection, which accounted for 43% of the subtotal. The cumulative rate of patients who received interferon therapy was 32%. Interferon therapy should be prescribed more frequently to HIV-positive patients with coagulation disorders in order to realize the survival benefits, although clinicians should be aware of side effects and toxicities.