Tetralogy of Fallot (TOF) is the most common form of cyanotic congenital heart disease. Long-term outcomes are favorable after corrective surgery, and 90% of patients reach adulthood. TOF is usually described as isolated obstructions of the right heart and are rarely accompanied by left-sided obstructive lesions such as cor triatriatum, mitral stenosis, subaortic or aortic stenosis, and aortic coarctation (1). In this report, we describe the presentation and treatment of an extremely rare case of TOF with left aortic arch and coarctation between the left common carotid and left subclavian arteries.