Familial hypercholesterolemia (FH) is also known as familial hyperbeta lipoproteinemia. Clinically characterized by hypercholesterolemia, characteristic yellow tumors, and a family history of early onset cardiovascular disease, FH is the most common inherited hyperlipidemia in childhood and the most serious of the lipid metabolism disorders that can lead to the development of a variety of life-threatening cardiovascular complications and is an important risk factor for coronary artery disease. Familial hypercholesterolemia is a rare autosomal dominant disorder with familial characteristics in which patients have abnormally high low-density lipoprotein (LDL) cholesterol values, usually 500-1200 mg/dL or even more than 700 mg/dL, if they are homozygous. But triglycerides are normal; also easy to develop cardiovascular disease early in life. Treatment 1. Diet control: Growing children should try to have less than 30% of daily calorie fat. A diet containing hydrolyzed fiber, fruits and vegetables is beneficial in lowering blood cholesterol. 2. Lipid-regulating drugs When children’s LDL-C exceeds 160 mg/dl (normal <110 mg/dl), careful evaluation is required and drug therapy is necessary to prevent the risk of cardiovascular disease. Familial hypercholesterolemia in pure congeners often requires further treatment when diet and medication are not effective due to genetic deficiencies. 3.Hemodialysis. 4.Liver transplantation.