Moyamoya disease is a cerebrovasculopathy of unknown etiology characterized by chronic progressive stenosis of the arteries of the circle of Willis. An extensive collateralized circulation forms, giving rise to the smoky appearance seen on cerebral angiography (moyamoya is Japanese for “puff of smoke”). Primary clinical features of moyamoya disease, especially in children, are related to cerebral ischemia and include transient ischemic attacks and stroke. The goals of treatment are primarily to restore blood flow to the affected areas, either by direct or indirect bypass, as there is no curative treatment for moyamoya disease. This chapter details the perioperative management of a patient with coexisting morbidities of moyamoya disease, coarctation of the aorta, and pulmonary hypertension, discussing the concerns related to each and their considerations in developing an anesthetic management plan.