Fourteen patients suspected of having Moya Moya disease underwent intravenous digital subtraction angiography (DSA), and the findings from nine of these patients were reviewed and analyzed. Obstruction or stenosis of the supraclinoid portion of the internal carotid artery and the proximal portions of the anterior and middle cerebral arteries was observed in all cases. Arteriography, however, was better than intravenous DSA in demonstrating Moya Moya vessels, differentiating complete occlusion from severe stenosis, and demonstrating important transdural collaterals. The disadvantages of intravenous DSA included inferior spatial resolution, the need for large amounts of contrast media, and relatively high radiation doses. Since the summation of vessels cannot be avoided using DSA, arteriography is necessary for the precise evaluation of cerebral hemodynamics before bypass surgery. Intravenous DSA is a safe, reliable method for diagnosing Moya Moya disease and is suitable for the screening and follow-up examinations of patients with the disease.