Moyamoya disease is an idiopathic vasculopathy, affecting vessels of Circle of Willis.1 It usually manifests as stroke, but can also cause seizures and cognitive impairment.2 Ischemic strokes are common in children and hemorrhagic strokes in adults.1 We describe our experience with moyamoya disease in four patients who presented with ischemic strokes, at an academic tertiary care center and emphasize that this diagnosis should be considered in young patients, especially children, who present with stroke.