Homozygous familial hypercholesterolemia (HoFH) is the rare and most severe form of FH. Those with HoFH usually have LDL cholesterol levels over 400 mg/dL and a family history of high cholesterol and/or early heart disease. Treatment for HoFH should begin as soon as possible, regardless of age. Anyone with HoFH, or suspected HoFH, should be seen by a lipid specialist.

A great deal of progress in HoFH treatment has been made in recent years. There are multiple effective treatments for HoFH available today. A combination of treatments is usually required to lower LDL cholesterol to recommended levels.