We report a case of a 35-year-old man with a dilated ascending aorta and a unique meandering retrosternal course of the right coronary artery (RCA) resulting in a partially empty right atrioventricular groove. The aortic root showed an exaggerated clockwise rotation, resulting in an anteriorly directed RCA ostium and the RCA, instead of entering the right atrioventricular groove, traversed caudally in the subepicardial space over the anterior surface of the right ventricle directly posterior to the sternum.