OBJECTIVE: The purpose of this study was to clarify the risk of rupture of unruptured intracranial aneurysms among large groups of patients with various underlying diseases or conditions. METHODS: A long-term follow-up study of unruptured intracranial aneurysms was performed with 360 patients who were treated conservatively during the period from April 1969 to December 1992. RESULTS: Follow-up evaluation (between February and June 1994) could be performed for 234 (65%) of the patients. The underlying diseases included multiple aneurysms with subarachnoid hemorrhage for 60 patients, cerebral infarction for 108, intracerebral hemorrhage for 27, and other diseases for 39. Single aneurysms were present in 171 patients and multiple aneurysms in 63. The mean follow-up period was 75 months (range, 3-270 mo). Of the 234 patients, 132(56.4%) survived, 59 (25.2%) died because of other diseases, 9 (3.8%) underwent surgery, and 34 (14.5%) showed bleeding from unruptured aneurysms, which was fatal for 18 of the patients. The average annual rupture rate for all patients was 2.3% (subarachnoid hemorrhage, 3.2%; cerebral infarction, 2.2%; intracerebral hemorrhage, 3.2%; other diseases, 3.6%). There were no significant differences among the patients according to underlying disease or aneurysm site. The cumulative rate of bleeding for all patients was 20% at 10 years after diagnosis and 35% at 15 years. The cumulative probability of rupture was significantly higher for the multiple aneurysms than the single aneurysms (P < 0.001). CONCLUSION: The risk of rupture of unruptured aneurysms is high, especially for multiple aneurysms, but there are no significant differences in the risk of rupture according to the underlying disease or the aneurysm location. Radical treatment should be considered for patients with unruptured intracranial aneurysms.