An 8-year-old girl presented with fibromuscular dysplasia of the intracranial vessels manifesting as ischemic stroke. Neuroimaging showed infarction of the right putamen and ipsilateral frontal white matter. Angiography revealed "string of beads" sign involving the terminal portion of the right internal carotid artery and the horizontal segment of the ipsilateral middle cerebral artery. She was treated conservatively. Magnetic resonance angiography at 2 months post ictus showed similar findings in the middle cerebral artery but improvement of the stenosis of the internal carotid artery. Her neurological deficits had almost resolved. Fibromuscular dysplasia should be part of the differential diagnosis of ischemia in children.