Abstract The presence of intracranial aneurysm in association with arteriovenous malformation has been well documented. Aneurysms have been described in typical proximal sites along the feeding system to the arteriovenous malformation, in abnormal distal locations along feeding vessels, and in sites remote and apparently hemodynamically unrelated to the arteriovenous malformation. Little attention has been focused on the most appropriate medical and surgical care of patients harboring these lesions. Since 1977, 22 patients with this combination of lesions have been evaluated at our institution. Nine patients (41%) presented after intracranial hemorrhage. The remaining 13 patients were investigated because of seizures in 5 patients (23%), headaches in 4 patients (18%), and progressive ischemia in 4 patients (18%). Among the patients suffering intracranial hemorrhage, 78% had bled from an aneurysm, with 22% having hemorrhaged from their arteriovenous malformation. All 7 of the patients who suffered aneurysmal hemorrhage bled from atypical distal aneurysms on major feeding vessels. Our experience and that of others has led us to believe that the safest approach to patients with this combination of lesions is to treat the aneurysm before microsurgical resection of the associated arteriovenous malformation. Hemodynamic changes associated with the abrupt elimination of an arteriovenous malformation may place associated aneurysms at immediate risk.