Congenital abnormalities of the coronary arteries are uncommon but can be associated with important cardiac events depending on their location and the course of the aberrant artery. Conventional angiography has been the gold standard for the diagnosis of these anomalies. The recent development of cardiac CT has allowed accurate and non-invasive depiction of coronary artery anomalies in terms of their origin, course and termination. We describe the case of a patient presenting with acute inferior myocardial infarction who was found to have a very rare congenital abnormality consisting of an anomalous origin of the right coronary artery from the mid-segment of the left anterior descending. Coronary angiography and cardiac CT angiographic images are shown and discussed.