To determine the incidence of associated carotid artery disease and the effect of carotid endarterectomy on subsequent neurologic sequelae, a retrospective study of 66 patients with central retinal artery occlusion (CRAO) was undertaken. Ipsilateral extracranial carotid artery disease was present in 23 of 33 patients (70%) who had carotid arteriography. Sixteen patients had carotid endarterectomy following their CRAO (Group I) and 50 did not (Group II). Seven of the 40 patients available for follow-up in Group II had a subsequent stroke (mean follow-up: 54 months). Of the seven Group II patients shown to have associated carotid disease (Group IIs), three (43%) had a subsequent stroke during follow-up (mean: 28.3 months) compared to zero in Group I (p = 0.033; mean follow-up: 18.7 months). Because of the strong association between CRAO and ipsilateral carotid artery disease and because of the significantly higher incidence of subsequent ipsilateral stroke in CRAO patients with carotid disease who did not undergo endarterectomy, thorough evaluation of the carotid arteries followed by carotid endarterectomy, if indicated, is warranted in CRAO patients who have no other obvious etiology for the occlusion.