Moyamoya disease is a rare disorder associated with progressive intracranial arterial stenosis with fragile, small collateralization that gives an angiographic appearance of a puff of smoker or, in Japanese, "moya-moya". We report a case of coronary artery ostial occlusive disease as an extracranial manifestation of Moyamoya. In the case, we demonstrate that thigh risk features of cardiac positron emission tomography (PET) that ultimately lead to the diagnosis of coronary artery occlusion.
