Moyamoya disease is characterized by the angiographic findings of bilateral occlusion of the distal portion of the internal carotid arteries with a vascular network at the base of the brain. The typical findings on conventional angiography and magnetic resonance imaging usually confirm the correct diagnosis of moyamoya disease. We experienced a 11-year-old girl with moyamoya disease showing repeated transient ischemic attacks and we report on this case with a review of the literature.