Background : The risks of recurrent ischemic stroke and acute coronary syndrome were increased in the large artery atherosclerotic subtype. The purpose of this study was to compare 2-year outcomes between ischemic stroke patients with intracranial arterial stenosis without significant extracranial carotid stenosis and patients with extracranial carotid stenosis. Methods : The author prospectively studied 123 ischemic stroke patients: 71 patients with intracranial arterial stenosis and 52 patients with extracranial carotid stenosis. Neurologic and radiologic investigations were performed at the beginning of the study. All of the patients were treated as regular out-patients of the neurology unit with a mean follow-up of 24 months. Recurrent stroke, myocardial infarction, and death were recorded. Results: Fifteen patients of extracranial carotid stenosis group and eighteen patients of intracranial arterial stenosis group developed recurrent stroke during follow-up (p = 0.049). Acute coronary syndrome occurred in eight patients of extracranial carotid stenosis group and only one of intracranial arterial stenosis group (p<0.05). Causes of death are end-stage cancers, stroke and related conditions, and acute coronary syndrome. The multivariate analysis showed symptomatic extracranial carotid stenosis is the important risk factor of acute coronary syndrome (p<0.05, RR = 12.8) Conclusion : There was no significant difference of recurrent ischemic stroke and recurrent stroke between patients with intracranial arterial stenosis without extracranial carotid stenosis and patients with extracranial carotid stenosis. On the other hand, patients with extracranial carotid stenosis significantly had more incidences of acute coronary syndrome than patients with intracranial arterial stenosis.