PURPOSE: State of the art of LDL-apheresis and treatment of severe familiar hypercholesterolemia. PATIENTS AND METHODS: Clinical experience of treatment with LDL-apheresis of pediatric and adult patients with familial hypercholesterolemia using the following techniques: D.A.L.I., H.E.L.P., D.S.C. RESULTS: The outcome of treatment with LDL-apheresis in young patients, using the most recently introduced techniques, is reported. We have submitted to LDL-apheresis 11 pediatric patients. The youngest is aged 3.5. LDL-apheresis is able at improving the metabolic impairment and halting the natural evolution of atherosclerotic disease and atherosclerotic complications. CONCLUSIONS: At present, LDL-apheresis is the most effective and safe therapeutic approach to the treatment of homozygous, heterozygous and double heterozygous familial hypercholesterolemia.