Central retinal artery occlusion usually results in blindness. The association between central retinal artery occlusion and extracranial carotid disease has not been clearly delineated. We reviewed the case reports of 62 patients with central retinal artery occlusion, 25 of whom underwent carotid angiography as part of the diagnostic evaluation. Fourteen of the 25 (56 percent) were found to have ipsilateral extracranial carotid disease. These patients did not generally have carotid bruits and had normal noninvasive carotid tests. Ten patients underwent ipsilateral carotid endarterectomy; these patients had either embologenic ulcerated plaque or tight stenosis of the carotid artery. There were 11 patients who showed no abnormalities on angiography. Thirteen patients who did not undergo angiography showed clinical evidence of etiologic factors, including vasculitis, an embolism of cardiac origin, and trauma. The remaining 24 patients had no diagnostic workup. Follow-up data were available in six of those patients who underwent carotid endarterectomy for a mean of 34 months; no strokes were reported. In conclusion, over half of patients with central retinal artery occlusion who undergo carotid angiography will have a carotid lesion on the ipsilateral side. This suggests that central retinal artery occlusion is a significant marker for extracranial carotid disease and should be an indication for complete carotid evaluation.