Familial hypercholesterolemia is a frequent genetic disorder characterized by elevated LDL-cholestrol and premature coronary heart disease. Familial hypercholesterolemia remains largely underdiagnosed in the general population and for many patients the initial clinical manifestation is acute coronary syndromes (ACS). Furthermore, many missed diagnosis of familial hypercholesterolemia can also occur during hospitalization for ACS. In this review, we aim to discuss the identification and prognosis of familial hypercholesterolemia after hospitalization for ACS.