Congenital coronary anomalies are uncommon but can cause sudden cardiac death or myocardial ischemia. Conventional coronary angiography is an invasive and expensive modality, sometimes unable to delineate the exact origin and course of an anomalous artery. In this case report, we describe an "extremely rare" anomaly of the right coronary artery where a 64-slice multidetector computed tomography provided valuable information regarding its exact site of the aortocoronary take-off and its spatial relationship with the great vessels. Knowledge of CT appearances and an understanding of the clinical significance of these anomalies are essential for making the correct diagnosis and planning patient treatment.