The authors conducted a long-term follow-up study of 168 patients to define the natural history of clinically unruptured intracranial arteriovenous malformations (AVM's). Charts of patients seen at the Mayo Clinic between 1974 and 1985 were reviewed. Follow-up information was obtained on 166 patients until death, surgery, or other intervention, or for at least 4 years after diagnosis (mean follow-up time 8.2 years). All available cerebral arteriograms and computerized tomography scans of the head were reviewed. Intracranial hemorrhage occurred in 31 patients (18%), due to AVM rupture in 29 and secondary to AVM or aneurysm rupture in two. The mean risk of hemorrhage was 2.2% per year, and the observed annual rates of hemorrhage increased over time. The risk of death from rupture was 29%, and 23% of survivors had significant long-term morbidity. The size of the AVM and the presence of treated or untreated hypertension were of no value in predicting rupture.