Familial hypercholesterolemia (FH) is a form of genetic dyslipidemia characterized by high levels of serum low-density lipoprotein cholesterol, tendon xanthomas, and family history of heart disease or elevated cholesterol. We report the case of a 24-year-old young male who presented with acute coronary syndrome, multiple skin and tendon xanthomas, family history of premature cardiac death and diagnosed as FH with coronary artery disease which was treated with percutaneous coronary intervention and lipid-lowering therapy.