**Abstract**

Moyamoya disease is a rare, progressive cerebrovascular occlusive disease; characterized by narrowing of the distal internal carotid arteries and their branches. The incidence is high in East Asians and most commonly presents in the first and fourth decade of life. Its symptoms are headaches, seizures, transient neurological deficits, and cognitive decline. Medical management is based on treating the symptoms and includes antiplatelet and anti-seizure medications. Surgical revascularization is the mainstay of treatment. Unique pathophysiology of moyamoya disease necessitates neuro-anesthesiologists to formulate an individualized plan perioperatively. The overriding goal of perioperative anesthetic management of moyamoya disease is to ensure optimal cerebral perfusion and protection. Maintenance of normotension, normocarbia, normo-oxygenation, normothermia, and euvolemia is the cornerstone during the perioperative period. Perioperative adequate analgesia is crucial to prevent cerebral ischemia and allows close neurological monitoring. This chapter reviews perioperative anesthetic management of patients with moyamoya disease.

**Keywords:** moyamoya disease, perioperative consideration, anesthetic management, indocyanine green, total intravenous anesthesia, scalp nerve block, special considerations in non-revascularization surgery
