OBJECTIVE To analyse whether contralateral occlusion represents an additional perioperative risk factor in carotid endarterectomy (CEA), and whether long-term survival after surgery in patients with contralateral occlusion differs from that of patients without. METHODS Retrospective clinical study. METHODS Vascular Surgery Unit, Department of Surgery, University of Perugia, Perugia, Italy. METHODS Fifty-five patients with carotid stenosis and contralateral occlusion undergoing CEA (Group 1) were compared with 110 patients (Group II), without contralateral occlusion selected from a cohort of 367 patients with a patent contralateral artery, matched for gender, age and ipsilateral symptoms. METHODS Perioperative stroke/death rate at 30 days and minor complications in Group I vs. Group II over a mean follow-up of 38 months. RESULTS The perioperative stroke/death rate at 30 days was 0% in Group I and 2.7% in Group II (p = 0.6) while minor complications amounted to 11% in Group I and 5% in Group II (p = 0.2). Survival rates of patients free from stroke, using Kaplan Meier curves, were 79.4% in Group I and 83.3% in Group II (p = 0.4); stroke free rates were 92.8% and 94.3% in Groups I and II, respectively. The incidence of late stroke, fatal or not, in patients who had undergone CEA with contralateral obstruction was the same as in similarly operated patients without contralateral obstruction (7% vs. 6%). However, the incidence of late vascular death, exemplified by a crude rate of 14% vs. 6% (p = 0.1; O.R. = 2.50; C.I. = 0.77-8.25) was greater in patients with contralateral occlusion. CONCLUSIONS In this study, CEA in patients with contralateral occlusion was not associated with an increased perioperative morbidity/mortality rate. The higher incidence of vascular death in the late follow-up of patients with contralateral carotid occlusion, although not statistically significant, could indicate the presence of more severe systemic vascular disease.