Familial hypercholesterolemia is a common genetic disorder. It is characterised by high cholesterol levels and early cardiovascular disease. With systematic biochemical testing in children, we can establish an early diagnosis of hypercholesterolemia. Furthermore, high risk individuals can be identified by measuring morphological and functional properties of arterial wall together with endothelial function assessment. In hypercholesterolemic children, early non-pharmacological (life-style change) intervention is effective and in high risk individuals pharmacological intervention can be timely and adequately introduced. These measures significantly reduce incidence of manifest cardiovascular disease in individuals with familial hypercholesterolemia and the related economic burden.