A 58-year-old Caucasian lady presented for severe left internal carotid artery (ICA) stenosis. Two months before she was operated for right carotid endarterectomy (CEA) in another Institution, complicated with internal carotid artery thrombosis and development of transient hemiplegia and aphasia. Postoperative selective DSA showed the development of an abnormal basal meshwork of collateral vessels with typical evidence for moyamoya disease. Preoperative workup in our Institution included cerebral MRI witch showed two ischemic right frontal and parietal lesions. The patient underwent successful stenting of the left ICA. This is the first report of extracranial ICA stenting in a patient with moyamoya syndrome.