Familial hypercholesterolemia is a rare inherited disease that results in elevated levels of low-density lipoprotein (LDL) and clinically leads to early coronary artery disease. It is rare for this disease to be associated with the coarctation of the aorta. In this report, we review the clinical case of an 18-year-old who was referred to our center with angina pectoris manifestation. A comprehensive clinical and investigative evaluation of the patient pre-operatively showed the presence of familial hypercholesterolemia, Coarctation of the aorta, and ischemic cardiomyopathy that required coronary artery bypass surgery.