Familial hypercholesterolemia (FH) is a genetic lipoprotein disorder characterized by elevations in low-density lipoprotein cholesterol (LDL-C) >95th percentile for age and gender. Affected individuals may show clinical manifestations (xanthomas, xanthelasmas and premature arcus corneus) although these manifestations are seen less frequently with early diagnosis and treatment. Untreated, FH will lead to atherosclerotic cardiovascular disease (CVD), primarily coronary artery disease (CAD). There are at least three accepted definitions for FH, based on absolute LDL-C levels (MedPed criteria), the Simon-Broome criteria include a fixed cut-point for LDL-C, the presence of DNA mutations and xanthomas and the FH Dutch Criteria (the Netherlands).