We examined a patient with central retinal artery occlusion (CRAO) who was diagnosed as having pseudoxanthoma elasticum and exhibited angioid streaks. Echocardiography revealed stenosis and plaques of the right carotid artery. Magnetic resonance imaging (MRI) showed multiple cerebral infarctions, which were considered to be the result of vascular-endothelial abnormalities associated with pseudoxanthoma elasticum. Systemic examination of any plaque which may cause CRAO is recommended.