Although persons with hemophilia are known to be at increased risk of death, no studies have examined the source of medical care and other personal characteristics for associations with mortality. To determine death rates and to identify causes of death and predictors of mortality, we studied a cohort comprised of all hemophilic males identified by a six-state surveillance system. Data were obtained by medical record review of contacts with physicians, hemophilia treatment centers (HTCs), and other sources of care during 1993-1995 and from death certificates. Factors examined included age, race, state of residence, health insurance type, medical care source, hemophilia type/severity, presence of inhibitor, liver disease, HIV infection, and AIDS. A total of 2950 subjects were followed for an average of 2.6 years. Their median age was 22 years; 73% were white, 79% had hemophilia A, 42% had severe disease, and 67% had visited an HTC. During 7575 person years (PYs) of observation, 236 persons died-an age-adjusted mortality rate of 40.4 deaths/1000 PYs; 65% of deaths were HIV related. In addition to age, factors independently associated with increased risk of death (relative risk, P value) were the following: AIDS (33.5, <.001); HIV infection (4.7, <.001); liver disease (2.4, <.001); and Medicare/Medicaid insurance (1.4,. 01). Those persons who had received care in an HTC had a significantly decreased risk of death (0.6,.002). Although HIV infection and the presence of severe liver disease remain strong predictors of mortality, survival is significantly greater among hemophilics who receive medical care in HTCs. (Blood. 2000;96:437-442)