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The treatment of hypercholesterolaemia in children is often discussed as part of the primary prevention strategy for premature coronary disease in adults. Cholesterol-lowering drugs are appropriate in children with hereditary autosomal dominant diseases such as familial hypercholesterolaemia, familial Apo B100 deficiency, or combined familial dyslipidaemia. Indeed, these diseases are associated with a high risk of cardiovascular attacks in young adults. In children suffering from these diseases, cholesterol-lowering drugs are considered when the plasma low density-lipoprotein (LDL)-cholesterol concentration remains above 190 mg/dL after a 6-month dietary treatment. The drug of first choice remains bile acid-binding resines (colestyramine) because their efficacy and safety are well documented in children. HMG-CoA reductase inhibitors can be used in children older than 8 or 9 years of age in cases of an altered observance of colestyramine treatment, but their long-term tolerance is unknown. Fibrates are also efficient, however, their safety has not been evaluated in controlled studies through in children.