Familial hypercholesterolemia (FH) is a genetic disease caused by a mutation in low-density lipoprotein (LDL) receptor gene. It causes various presentations including tendon xanthoma and cardiac manifestations. Herein, we present a young patient with homozygous FH (HFH) who presented with dyspnea and chest pain caused by coronary arteries stenosis and treated with coronary artery bypass graft (CABG) surgery at the age of 13 years. To the best of our knowledge, he is one of the youngest patients in the English language literature for whom coronary revascularization has been done in childhood.