Moyamoya disease is a progressive cerebrovascular disorder with stenosis or occlusion of the bilateral internal carotid arteries with abnormal vascular networks at the base of the brain. Previous reports have shown that there are extracranial vascular involvements in Moyamoya disease, especially in the renal artery. We report a 7-year-old patient with Moyamoya disease associated with renovascular hyper tension, who presented in infancy with seizures and hemiparesis. Renal angiography showed multiple stenoses of the right renal artery. Although renal artery stenosis in Moyamoya disease has been effectively treated with balloon angioplasty, stent implantation, or surgery, bat-loon angioplasty could not be done in this patient due to multiple stenoses. His blood pressure was successfully controlled with medical treatment, and remained normotensive during the follow up period of 6 months.