Our purpose was to assess the value of three-dimensional (3D) CT angiography in the diagnosis of moyamoya disease. We studied seven patients with moyamoya disease proved by conventional angiography. Three-dimensional (3D) CT angiography was performed using rapid sequence or helical (spiral) scanning in conjunction with a bolus injection of intravenous contrast medium. All seven patients could be diagnosed as having moyamoya disease on the basis of the following 3D CT angiographic findings: poor visualisation of the main trunks and/or major branches of anterior and middle cerebral arteries (7 patients); dilated leptomeningeal anastomotic channels from the posterior cerebral arteries (4); and demonstration of "moyamoya vessels" in the basal ganglia (2). Although conventional angiography remains the principal imaging technique for demonstrating anatomical changes in detail, less invasive 3D CT angiography provides a solid means of diagnosing moyamoya disease when it is suspected on CT, MRI, or clinical grounds.