Background Moyamoya disease (MMD) is a chronic and progressive cerebrovascular occlusive disease with special pathophysiology features: cerebral perfusion pressure goes down and cerebral blood flow decreases gradually along with the development of the disease in the anterior circulation; cerebrovascular reserve is abated, the response to the carbon dioxide is unidirectional; the autoregulation of CBF to the ischemic cerebral areas is impaired and the CBF is totally pressure dependent. Objective Summarizing the features of anesthetic management for MMD is to provide reference data for clinical work. Content We have reviewed the aspects that include the pathophysiology of MMD, preanesthetic preparation, anesthetic induction and maintainance and prophylactic measures for cerebral ischemia so on. Trend With the medical technology progress, MMD detection rate is increased significantly, and surgical treatment is improved gradually. Positive and cautious anesthetic managements are the key to keep balance between anterior circulation cerebral oxygen supply and demand, and to prevent cerebrovascular accident.