Moyamoya syndrome has been rarely associated with Down syndrome. In pediatric patients, the usual presentation is the ischemic stroke. We report a 8 year old girl with Down syndrome and Moyamoya syndrome, who presented with focal seizure and acute left onset hemiparesis. The aim of this study is to describe the clinical and radiological features of the patient, the managment and the possible causes that could explain these two syndrome's associations have been reviewed.
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