Spontaneous occlusion of the circle of Willis (moyamoya disease), a rare ce
rebrovascular disease, may lead to repeated hemodynamic stroke due to unrem
itting changes of the basal cranial vessels. Moyamoya can be diagnosed on i
ts specific epidemiological, clinical,and radiological features. In patient
s with distinctive Moyamoya disease, improvement of cerebral blood supply b
y means of neurosurgical revascularization enables definitive treatment. Th
is review summarizes current knowledge of the clinical and diagnostic featu
res of this disease and illustrates its pathological and pathophysiological
characteristics. Additionally, indications and techniques of current neuro
surgical revascularization procedures are illustrated.