The authors have updated a series of 166 prospectively followed unoperated symptomatic patients with arteriovenous malformations (AVM's) of the brain. Follow-up data were obtained for 160 (96%) of the original population, with a mean follow-up period of 23.7 years. The rate of major rebleeding was 4.0% per year, and the mortality rate was 1.0% per year. At follow-up review, 23% of the series were dead from AVM hemorrhage. The combined rate of major morbidity and mortality was 2.7% per year. These annual rates remained essentially constant over the entire period of the study. There was no difference in the incidence of rebleeding or death regardless of presentation with or without evidence of hemorrhage. The mean interval between initial presentation and subsequent hemorrhage was 7.7 years.