Atherosclerosis represents a disease that begins in childhood, and alterations in lipid concentration play a fundamental role in the development of this condition. Children and adolescents with high cholesterol levels are more likely than their peers in the general population to present with dyslipidemia in adulthood. Precocious identification of dyslipidemias associated with premature cardiovascular disease is crucial during childhood to delay or prevent the atherosclerotic process. The National Cholesterol Education Program has established guidelines for the diagnosis and treatment of dyslipidemia during pediatric age. It has been suggested that a heart-healthy diet should begin at 2 years of age, and no adverse effects on psychological aspects, growth, pubertal development and nutritional status in children and adolescents limiting total and saturated fat intake have been demonstrated. Pharmacotherapy should be considered in children aged 10 years or older when low-density lipoprotein cholesterol concentrations remain very high despite dietary therapy, especially when multiple risk factors are present. The lipid-lowering drugs recommended for childhood and adolescence are resins and statins. The increasing use of statins is dependent on their effectiveness and safety. Ezetimibe, a selective cholesterol absorption inhibitor, may provide a similar cholesterol-lowering effect as that reached with statin treatment. This review provides an update on recent advances in the therapy of dyslipidemia, especially hypercholesterolemia, during pediatric age and adolescence.