The purpose of this study was to determine the symptoms at presentation and the incidence of intracranial hemorrhage (ICrH) caused by intracranial vascular malformations (IVMs) in a defined population. The authors used the Mayo Clinic medical records linkage system to detect all cases of IVM among residents of Olmsted County, Minnesota, during the period 1965 through 1992. Forty-eight IVMs were detected over the 27-year period. Twenty-nine of the 48 patients were symptomatic at presentation. The most common presenting symptom was ICrH, which was present in 20 patients, 69% of all symptomatic cases. Sixty-five percent of arteriovenous malformations (AVMs) presented with ICrH. The most common subtype of ICrH was intracerebral hemorrhage, which was found in nine of 20 patients; the second most common subtype was subarachnoid hemorrhage. The peak occurrence of hemorrhage was during the fifth decade of life. The age- and gender-adjusted occurrence of a first ICrH from an IVM among residents of Olmsted County, Minnesota was 0.82 per 100,000 person years (95% confidence interval 0.46-1.19). There was no increase in the detection of IVM-related ICrH throughout the study period. The 30-day mortality rate following ICrH was 17.6% for patients with an AVM and 25% for all patients with IVMs. This study provides unique data on symptoms at presentation and the incidence of ICrH and hemorrhage subtypes from IVMs on a population basis.