Homozygous familial hypercholesterolemia (FH) is an autosomal dominant disease caused by a mutation in the low-density lipoprotein (LDL) receptor. This mutation can lead to increased serum LDL, and subsequently to premature coronary artery disease. It may also lead to valvular and supravalvular aortic stenosis, these complications being cardinal in the natural course of the disease. The surgical treatment of aortic stenosis in patients with homozygous FH is accompanied by high risk, even in skillfu1 hands. Herein is presented the long-term follow up of a young patient with homozygous FH who underwent coronary artery bypass graft surgery at the age 14 years. Although the patient developed aortic stenosis five years later, neither the native coronary vessels nor grafted vessels underwent any atherosclerotic changes during this period.