This case shows typical Moyamoya disease with recurrent bilateral cerebral infarctions and brain atrophy. CT angiography shows progressive attenuation of the distal ICAs and the proximal circle of Willis, with collateralization through hypertrophied lenticulostriate and thalamostriate arteries. The word Moyamoya is a Japanese word that means a puff or spiral of smoke describing the cloud-like lenticulostriate and thalamostriate collaterals on conventional or CT angiography.