Anomalous origin of the right coronary artery is an infrequent anomaly. A case with anomalous origin of the right coronary artery from the ascending aorta above the left sinus of Valsalva is described. This patient was also found to have a bicuspid aortic valve. The aortic valve was markedly calcified and stenotic. The aortic valve was replaced with a St. Jude Medical prosthesis.