Anomalous Aortic Origin of Coronary Artery is rare congenital anomaly seen in 0.1 – 0.2 % of general population. Situs inversus with dextrocardia is known as situs inversus totalis. Authors report a case of anomalous aortic origin of right coronary artery from left sinus of Valsalva in a patient with situs inversus totalis , who also had other anomalies like ventricular septal defect and persistent superior vena cava.