According to current literature, there is no clinical report regarding coronary spasm in the presence of aortic coarctation. There are no cases in which endothelial function was restored after the repair of aortic coarctation. A 29-year-old woman with aortic coarctation and acute myocardial infarction due to coronary spasm experienced restoration of endothelial function after the repair of aortic coarctation. This case may increase knowledge of the mechanism of coronary damage in human coronary arteries not only with a coarcted aorta but also with hypertensive heart.