Bilateral internal carotid artery occlusion (BICAO) is a rare disease that carries a gloomy prognosis. We report a case of a 52-year-old man who developed ischaemic infarction at the region of the right middle cerebral artery; he was found to have atherosclerotic occlusion of both internal carotid arteries on Doppler-duplex examination. He received medical treatment only. After 1 year, he developed a new infarction at the region of the left middle cerebral artery. Conventional angiography revealed bilateral occlusion of internal carotid arteries at their origin, approximately 50% stenosis of the common carotid bulbs and mild stenosis of the origin of external carotid arteries. The patient did not undergo any form of surgical revascularisation procedures and died of severe aspiration pneumonia approximately 2 months after the second stroke. BICAO portends a poor outcome and carries a risk of recurrent ischaemic events. The best management strategy for this vascular occlusion remains unclear.