We describe the rare case of a 10-month-old girl who had coarctation of the aorta in association with tetralogy of Fallot. The surgical management and postoperative course is described. This case highlights the rare association of coarctation with tetralogy of Fallot, with a large intracardiac right-to-left shunt. Although an exception to the rule, it challenges the reduced fetal blood flow theory and the smooth muscle cell migration theory as embryological explanations for the development of coarctation.
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