Right aortic arch (RAA) and retro-aortic innominate vein are rare vascular anomalies. Diagnosis of these anatomical variations can be achieved using fetal echocardiography, post-natal echocardiography, and computed tomography scan. RAA can form a vascular ring when associated with other vascular anomalies which may compress the trachea and/or esophagus. On the other hand, the existence of retro-aortic innominate vein can influence the clinical decision-making and surgical strategy. We report a rare occurrence of both RAA and retro-aortic innominate vein in a 3 months old girl with a prenatal diagnosis of tetralogy of Fallot and include details of her presentation and successful management.