Many individuals with hemophilia were infected with human immunodeficiency virus (HIV) in the early 1980s through contaminated blood products. Most also were co-infected with hepatitis C virus (HCV). Deaths among the entire cohort of HIV-positive hemophiliacs in Canada up to 2003 are described. Using registry data, we analyzed Kaplan-Meier survival curves, determined the effect of age at HIV seroconversion on mortality, and described cause-specific proportional mortality patterns over time. Of 2427 Canadians with hemophilia, 660 (27.2%) were HIV-positive, of whom 406 (61.5%) died. In contrast, 114 (6.5%) deaths occurred in HIV-negative controls. Median age at HIV seroconversion was 20 (range, < 1-67 years), and median survival was 15.0 years (95% confidence interval, 13.6-16.4 years). Younger age at HIV seroconversion was associated with improved survival; however, this finding was not explained by differences in causes of death across age groups. Following the introduction of highly active antiretroviral therapy, the proportion of deaths due to acquired immune deficiency syndrome has decreased, while the proportion of deaths due to liver disease has increased. There were 1134 HCV-positive individuals, of whom only 444 (39.2%) were also HIV-positive. Liver disease is a growing health concern among many hemophiliacs, not only those who are HIV-positive.