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Background: Progressive stenosis of the supraclinoid segments of the internal carotid arteries, followed by formation of characteristic collateral brain circulation is typical for moyamoya disease. This illness, with unknown pathogenesis, is often diagnosed in Asiatic population. In Poland it can be a rare cause of ischemic infarcts in children. Case report: Two cases of cerebral ischemic infarct due to moyamoya disease in girls aged 7 and 12 are presented. The final diagnosis was established after MR exams and digital subtraction angiography (DSA) of the cerebral arteries. Conclusions: In spite of rare incidence, the moyamoya disease should be considered as the potential cause of cerebral ischemic infarctions in children. Despite the main role of the DSA in establishment of the final diagnosis, noninvasive neuroradiological examinations become more and more important in diagnostic schedule of moyamoya disease. MR and MR angiography visualize characteristic radiological symptoms, enabling preliminary diagnosis and are the method of choice in control examinations.