Moyamoya disease is a cerebrovascular disorder of unknown etiology that is characterized by bilateral progressive stenosis of the distal internal carotid artery and proximal anterior and middle cerebral arteries, which may result in transient ischemic attacks or strokes.1 Although it is not primarily an eye disorder, multiple ocular conditions including morning glory optic disc anomaly, chorioretinal coloboma, anterior ischemic optic neuropathy, ocular ischemic syndrome, and retinal vascular occlusions among others have been associated with the disease. We report a case of moyamoya disease in a young woman with unique retinal arterial vascular changes.