This report describes the first two Japanese cases of AIDS developed in patients with hemophilia. One was a 48-year-old man with hemophilia B and the other was a 62-year-old patient with hemophilia A. They had nonspecific prodromal syndromes of AIDS initially, and later developed fatal opportunistic infections with Candida or atypical mycobacteriosis and Aspergillosis. They had marked cellular immunodeficiency, and antibodies to HTLV-III. Subsequently, three more cases of AIDS in hemophiliacs have been reported in Japan. Because most of the clotting factor concentrates for treatment of hemophilia are imported from the United States and European countries, hemophiliac patients in Japan are also at high risk for AIDS.