The finding of carotid stenosis contralateral to a carotid occlusion is becoming more frequent. While the neurologic outcomes in this patient population have been described, the rate of disease progression measured by duplex examination and the eventual need for carotid endarterectomy has not been described. In this study, a computerized database of carotid duplex examinations was reviewed and clinical data were obtained from clinic records. From 9124 studies 117 patients were identified. Thirty patients had previous carotid surgery on the patent side and were excluded. Of 87 patients 33 required carotid endarterectomy on the patent side. The rate of disease progression and/or the performance of a carotid endarterectomy by life-table analysis was 85.9% over 8 years. There were 10 neurologic events during the follow-up period. Patients with carotid stenosis and contralateral occlusion are at significant risk for disease progression. Follow-up should be more frequent and of longer duration in this patient population. A significant number of patients with carotid artery occlusion will require a carotid endarterectomy of the patent contralateral carotid.