All causes of cerebrovascular disease should be considered in any patient presenting with ischemic stroke, despite the patient have concomitant risk factors for atherosclerosis. Here, we reported a case of Moyamoya disease (MMD) at a rather old age and with the presence of multiple risk factors for atherosclerosis. It is difficult to consider MMD at first glance, and it is easy to choose the wrong treatment in the first place. The key radiological feature for differentiation is the bilateral intracranial stenosis, rather than atherosclerotic changes, which are characterized as plaques with an uneven surface.