The one-year incidence of carotid occlusion is 6/100 000 inhabitants in general population. Stroke incidence and mortality rate in these patients vary. Patients that underwent carotid endarterectomy (CES) are at a higher risk of progression of contralateral carotid stenosis. The aim of the study was to investigate the management and natural history of the contralateral internal carotid artery disease in patients with internal carotid artery occlusion (ICAO). During one year, 297 patients with ICAO were investigated. Follow up examinations were retrospectively analyzed and patients were divided into groups according to contralateral carotid disease. Out of 297 patients, only one investigation was performed in 90 patients with carotid occlusion. Thirty three patients were followed up due to postoperative ICAO. In 14 patients, ICAO developed during ultrasonographic follow up. In this group of patients, 9 had unchanged contralateral findings, whereas in 5 patients disease progression was observed. Out of 44 patients with ICAO and contralateral subtotal stenosis at initial investigation, 42 underwent carotid surgery. Postoperatively, 32 patients had normal findings, 6 developed mild carotid stenosis, 2 developed moderate carotid stenosis, and 2 had postoperative carotid occlusion. Two patients were followed-up without intervention. Nine patients with bilateral ICAO were followed-up for years. Follow up was continued in 106 patients with ICAO and contralateral mild to moderate changes. The finding was unchanged in 68 patients. In 21 (30%) patients the disease progressed to subtotal stenosis and 18 patients underwent carotid surgery. Accordingly, contralateral carotid disease progression was observed in one third of patients with carotid occlusion. Additional studies on the issue are needed.