Familial hypercholesterolemia (FH) is a dominantly inherited disorder caused by mutation at the locus for the low-density lipoprotein (LDL) receptor and is frequently associated with premature coronary artery disease and aortic valve involvement. The surgical treatment of these complications is accompanied by a high degree of risk, even in skillful hands. An intensive cholesterol-lowering therapy and LDL aphaeresis in association with surgery may be useful. The case of a 12-year-old girl, with a medical history of familial hypercholesterolemia is reported here, operated two years previously for valvular aortic stenosis; Ross intervention was done. She was readmitted for acute coronary syndrome. Three coronary artery bypass grafting was performed with saphenous veins with positive results.