Moyamoya disease （MMD） is a chronic progressive cerebrovascular disease involving stenosis or occlusion at the terminal portion of the internal carotid artery and/or its proximal branches， leading to the formation of networks of collateral vessels. These changes cause chronic ischemia of the brain parenchyma and subsequent severe cerebrovascular accidents. Without proper treatment， adult MMD patients may gradually show the involvement of cognitive function， and the mortality rate of adult MMD patients is twice that of pediatric MMD patients. Due to the complex etiology and severe consequences of MMD， the treatment of this disease is particularly difficult and urgent. Surgical revascularization， as the cornerstone of MMD treatment， is classified into the three main categories of direct revascularization， indirect revascularization， and combined revascularization. Considering that indirect revascularization cannot reduce the incidence rate of perioperative stroke， direct revascularization is often selected as the preferred surgical procedure for patients with ischemic MMD. In order to prevent recurrent hemorrhage in patients with hemorrhagic MMD， direct revascularization or combined revascularization can achieve the goal of treatment by establishing collateral circulation and promoting revascularization. When patients develop new-onset ischemic stroke after surgery， indirect revascularization should be selected first. For hemodynamically unstable adult MMD patients， direct revascularization or combined revascularization should be the preferred choice. As there are currently no specific drugs to reverse the progression of MMD， medical therapy is limited to symptomatic treatment and perioperative management. In clinical practice， aspirin is often used to inhibit platelet aggregation， erythropoietin and statins are used to promote the development of collateral vessels， and dexamethasone is used to promote neovascularization. The efficacy of remote ischemic conditioning in improving the sequelae of MMD patients has been recognized by the industry， and in-depth research on pathogenesis may provide more possibilities for the diagnosis and treatment of MMD. Abnormal methionine cycle is involved in the pathogenesis of MMD， suggesting that methionine cycle-related risk scores have a good ability in predicting the risk of MMD. In addition， endothelial progenitor cell transplantation may become a new strategy for the treatment of MMD in clinical practice. ［Journal of International Neurology and Neurosurgery, 2023, 50(3): 78-83］