Abstract
Moyamoya disease is a progressive vascular disease associated with steno-occlusion of the terminal portions of the internal carotid arteries and with several congenital and acquired conditions, including ischemic stroke in young children. This case report describes the discovery of an unusual association between moyamoya disease and type 1 diabetes mellitus in a 9-year-old child presenting with stroke-associated diabetic ketoacidosis. Discovery of this case highlights the importance of considering stroke as well as cerebral edema, the more likely etiology, in the differential diagnosis of neurological deterioration in children with diabetic ketoacidosis, as well as considering moyamoya syndrome in the diagnosis of diabetic children presenting with stroke.