Familial hypercholesterolemia (FH) is an autosomal dominant inherited metabolic disorder, patient with FH has an elevated serum low density lipoprotein cholesterol(LDLC) and a high incidence of premature atherosclerotic cardiovascular disease (ASCVD). If untreated, patients with homozygous familial hypercholesterolemia (HoFH) develop premature death by the age of 30 years, generally from ASCVD. Cholesterol-lowing therapies for HoFH include therapeutic lifestyle changes, lipid-lowing pharmacologic therapy, lipid apheresis and liver transplantation, etc. However, the curative effect of the traditional lipid-lowering drugs is still very limited in HoFH population. It is difficult to carry out lipid apheresis constantly because of its high cost, invasiveness, long-term maintenance at weekly or biweekly interval and poor adherence. Nearly 90% of FH is caused by mutations in the LDL receptor (LDLR) gene. Since LDLR are located mainly in the hepatocytes, liver transplantation achieves the replacement of dysfunctional hepatic LDLR, and it is considered to be the effective way to correct hepatic cholesterol metabolism. This paper mainly review the efficacy and safety of liver transplantation, current challenges and the prospect of liver transplantation for HoFH.