Cardiac manifestations and angiographic characteristics of coronary artery disease in identical twins have been previously dealt with in a handful of case reports and series; yet, the results were highly controversial. Our rare case demonstrates striking similarities in both the timing and type of clinical manifestation, as well as in the underlying anatomy and the distribution of coronary artery disease. When premature coronary artery disease is found in one of a monozygotic twin pair, evaluating the other twin is a reasonable approach.