Chest pain in a young person without cardiovascular risk factors is usually attributed to noncoronary causes; however, if the history suggests ischemic pain, the potential presence of unusual cardiovascular abnormalities should not be disregarded. The present case describes a young man with solitary congenital ostial atresia of right coronary artery, who to our knowledge is only the second case in the medical literature. Manifestation of ischemic symptoms in a relatively advanced age in patients with coronary artery atresia may mislead clinicians to interpret them as signs of atherosclerotic coronary artery disease. Therefore congenital coronary artery atresia should be a part of the differential diagnosis particularly in young patients with ischemic symptoms and no cardiovascular risk factors. (C) 2009 Japanese College of Cardiology. Published by Elsevier Ireland Ltd. All rights reserved.