Objective: Intracranial arteriovenous malformation (AVM) associated with aneurysm has been infrequently encountered and the treatment for this malady is challenging. We report here on our clinical experience with AVMs associated with arterial aneurysms that were managed by multimodality treatments, including clipping of the aneurysm, microsurgery, Gamma-knife radiosurgery (GKS) and Guglielmi detachable coil (GDC) embolization. Methods: We reviewed the treatment plans, radiological findings and clinical courses of 21 patients who were treated with GKS for AVM associated with aneurysm. Results: Twenty-seven aneurysms in 21 patients with AVMs were enrolled in this study. Hemorrhage was the most frequent presenting symptom (17 patients: 80.9%). Bleeding was caused by an AVM nidus in 11 cases, aneurysm rupture in 5 and an undetermined origin in 1. Five patients were treated for associated aneurysm with clipping followed by GKS for the AVM and 11 patients were treated with GDC embolization combined with GKS for an AVM. Although 11 associated aneurysms remained untreated after GKS, none of them ruptured and 4 aneurysms regressed during the follow up period. Two aneurysms increased in size despite the disappearance of the AVM nidus after GKS and then these aneurysms were treated with GDC embolization. Conclusion: If combined treatment using microsurgery, GKS and endovascular treatment can be adequately used for these patients, a better prognosis can be obtained. In particular, GKS and GDC embolization are considered to have significant roles to minimize neurologic injury.