Chronic occlusive arterial disease of the periphery is primarily caused by atherosclerotic disease. In young patients with no identifi able risk factors for atherosclerosis, who present with symptoms of claudication or critical ischemia, other rare causes need to be suspected. Cystic adventitial disease is one such condition affecting young healthy patients. Although it has been reported most commonly in relation to the popliteal artery, other sites including the iliac artery can also get affected. Isolated short segment stenosis or occlusion can lead to signifi cant disabling symptoms restricted to one side. Imaging studies show pristine arterial anatomy with no evidence of systemic atherosclerotic disease and an isolated area of luminal stenosis. Defi nitive treatment involves open surgical excision with interposition grafting for optimal long-term results. We report a case of cystic adventitial disease affecting the external iliac artery in an otherwise healthy young man.