A SEVERE DISEASE: Homozygote hyper cholestrolemia is a very severe disease with an extremely high atherogenic potential. The risk of sudden death starts at about the age of 10 years and mortality reaches nearly 100% at 20 years. In this homozygote autosomal dominant disorder, serum cholesterol rises above 6 g/l due to a total deficiency of LDL-receptors. LDL-APHERESIS: The treatment of choice, LDL-apheresis, is an aggressive treatment. Drug regimens and palliative surgery have little or no effect. Liver transplantation is highly effective but compromises long term prognosis. LDL-apheresis, indicated in case of drug resistance, can be used to clear atherogenic LDL particles extracorporally. Currently it is the most effective means of lowering serum cholesterol in these patients and avoid potential cardiovascular complications. SEVERAL TECHNIQUES: The most specific techniques are also the most costly. These techniques allow treating whole blood or previously separated plasma. Such specific techniques are indicated in children. Compared with other European countries such as Germany, the development of these techniques has been retarded in France due to the lack of financing by the national health insurance system.