Moyamoya disease (MMD) is a complex cerebrovascular disorder about which little is known. Conventionally, revascularization surgery is recommended for patients, despite an absence of conclusive data from adequate clinical trials. Underscoring the uncertainty that exists in treating MMD patients, investigators continue to present data comparing revascularization with conservative or medical management, most of which originates from East Asia where MMD is most prevalent. The purpose of this manuscript is to review contemporary large case series, randomized trials, and recent meta-analyses that compare surgical and medical treatments in adult patients with MMD, and to critically analyze the modern literature in the context of current practice standards. Data from the available literature is limited, but revascularization seems superior to conservative therapy in adult patients presenting with hemorrhage, and in preventing future hemorrhages. Conversely, evidence that surgery is superior to medical therapy is not convincing in adult patients presenting with cerebral ischemia, or for the prevention of future ischemic events. In contrast to East Asian populations, MMD in Europe and in the Americas is predominantly an ischemic disease that presents in adulthood. Adequate multinational trials are warranted.