Some of hypercholesterolemias observed in childhood have a high risk of premature cardiovascular diseases. The monogenic dominantly inherited hypercholesterolemias such as the familial hypercholesterolemia due to mutations on LDL receptor gene corresponds to these diseases. This article, jointly elaborated by the Nouvelle Société Française d’Athérosclérose together with the Nutrition committee of the Société Française de Pédiatrie, is to propose recommendations for a screening strategy and for management of hypercholesterolemia in children. The approach of these high-risk inherited hypercholesterolemia is specified and the dietary management, the indications and supervision of lipid lowering drug therapy in children are discussed.