ABSTRACT Objectives To explore clinical features and compare of the difference between conservative treatment and surgical treatmentin prognosis of intracranial aneurysms associated with moyamoya disease (MMD). Methods 104 patients with MMD a ssociated with intracranial aneurysms diagnosed by digital subtraction angiography (DSA) or computed tomography angiography (CTA) were retrospectively analyzed. Results All patients of MMD with intracranial aneurysms had 129 aneurysms distributed at different sites. The distribution of the aneurysms was as follows: 28(21.71%) anterior communicating artery aneurysms, 21(16.28%) basilar artery aneurysms, 19(14.73%) middle cerebral artery aneurysms, 17(13.18%) posterior cerebral artery aneurysms, 12(9.30%)internal carotid artery aneurysms, 11(8.53%) posterior communicating artery aneurysms, 10(7.75%) anterior cerebral artery aneurysms, 5(3.88%) anterior choroidal artery aneurysms, 2(1.55%) moyamoya vessel aneurysms, and 4(3.1%) other location aneurysms. 82 cases (78.85%) had one aneurysm, and 21 cases (20.19%) had 2 or more aneurysms. 64 cases were treated surgically, and 40 cases were treated conservatively. 57 of 81 cases (70.37%) had a good outcome, 2 cases (2.47%) were in paralyzed, and 22 cases were died. The mortality rate was (27.16%). Death occurred in 16 (47.06%) of 34 patients with conservative treatment, and 6(12.77%) of 47 patients with surgical treatment. 7 cases (8.64%) had twice cerebral hemorrhage, and one case (1.23%) had third cerebral hemorrhage in the follow-up period. There were 5 deaths, two good outcomes, and one coma among 8 cases experienced re-hemorrhage. Conclusion Compared with conservative treatment, positive surgical treatment showed clinical significance for preventing cerebral hemorrhage and reducing mortality of MMD with intracranial aneurysms. Abbreviations MMD: moyamoya disease; DSA: digital subtraction angiography; CTA: computed tomography angiography