ABSTRACT. One hundred and eighteen patients with hemophilia A or B died in Sweden between 1957 and 1980. The causes and circumstances of death have been the subject of a retrospective study. The most striking feature was the increase in age at death. In severe hemophilia cases the age increased from 19 in the first half of the period to 50 in the second. In moderate and mild hemophilia cases the increases were similar, although less pronounced. The main cause of death was intracranial hemorrhage, which accounted for one-third of all deaths. In the last 12-year period, however, no patient below the age of 45 had succumbed to this. Age-related causes of death, such as malignancy and ischemic heart disease, had increased, as was to be expected from the increase in the mean and median ages of living hemophiliacs. The proportion of deaths unrelated to hemophilia was 36%. Antibodies were found in 5 (16.7%) of the patients with severe hemophilia A.