We evaluated the feasibility of endovascular embolization for the management of distal anterior choroidal artery (AChA) aneurysms associated with moyamoya disease and performed a literature review to summarize their clinical features and treatment. We describe two cases of moyamoya disease-associated distal AChA aneurysms treated by endovascular embolization. In both cases, a good outcome was observed. We performed a MEDLINE (1980–2010) search which identified 13 similar cases. Our analysis of the clinical data from these 15 cases led us to conclude that (i) endovascular embolization is an effective and feasible treatment for distal AChA aneurysms associated with moyamoya disease; (ii) aneurysm location and the preservation of the parent artery are two major prognostic factors for moyamoya disease-associated distal AChA aneurysms subjected to craniotomy or endovascular therapy; (iii) the parent artery should be preserved when the aneurysm is located in the temporal horn of the lateral ventricle, but sacrificed when it is located in the trigone of the lateral ventricle.