An isolated left subclavian artery is a rare anomaly. We report a 9-month-old boy with an isolated left subclavian artery associated with tetralogy of Fallot and the right aortic arch. MRI and angiography show that the blood supply through the left subclavian artery was maintained by a large tortuous collateral artery from the abdominal aorta. This type of collateral artery structure is unique.