Isolated RSCA from the right pulmonary artery is a very rare vascular arch anomaly. Complete loss of continuity of subclavian artery from the aortic arch was named ISOLATION by Stewart and colleagues [4] in 1964. They are commonly associated with DiGeorge syndrome [2] or intracardiac anomalies [3]. We report a case of 6 years old female child diagnosed with Tetralogy of Fallot with single pulmonary (right) artery with no left pulmonary artery with an anomalous Right Subclavian artery arising from the single pulmonary artery with left arch and left superior vena cava. Patient underwent single lung repair with total transatrial approach with reimplantation of the right subclavian artery into the right carotid/ innominate artery (first branch of the ascending aorta). Early intervention with complete surgical repair normalised the patient’s circulation with resolution of both the pulmonary and subclavian steal phenomenon.