A previously healthy 4-year-old female presented with an 18-month history of frequent headaches and seizures. Magnetic resonance imaging (MRI) and angiography revealed severe stenosis of the left proximal intracranial carotid artery, with bilateral development of moyamoya vessels, left greater than right. A diagnosis of moyamoya disease was made, and the patient was scheduled for surgical correction consisting of an encephalo-duro-arterio-synangiosis. Moyamoya disease is a rare, occlusive cerebrovascular disorder characterized by bilateral stenosis of the internal carotid arteries and their branches. While its etiology is currently uncertain, recent studies indicate that focal arteritis, secondary to an antigen-antibody reaction, leads to the development of the stenosis. Anesthetic management of patients with moyamoya disease focuses on maintenance of adequate cerebral blood flow, normalization of intracranial pressure, and avoidance of both cerebral vasoconstriction and vasodilation. Several anesthetic techniques have been successfully employed; one such method is presented.