A 53-year-old man with exercise-induced ischemia was referred for investigation. Coronary angiography revealed a right coronary artery originating from the trunk of the left coronary artery, and an aortic coarctation was observed on aortography. A CT angiogram confirmed these findings. Resection of the aortic coarctation and reimplantation of the ostial portion of right coronary artery into its native site was performed, and resulted in a satisfactory outcome. The association of an anomalous right coronary artery with aortic coarctation has rarely been described and represents a critical situation where early diagnosis and prompt intervention are essential.