For clinical neonatal hypercholesterolemia, further investigations such as liver ultrasound, liver function and virus-related tests should be performed first to clarify the cause. For viral hepatitis, the specific type of virus should also be clarified as much as possible and the appropriate treatment should be taken. For example, for hypercholesterolemia caused by cytomegalovirus infection, ganciclovir may be considered for antiviral treatment. If the hypercholestasis is caused by long-term intravenous nutrition, the amount of intravenous nutrition should be gradually reduced and replaced by nasojejunal drip enteral nutrition emulsion. Bile-reducing drugs can also be used, such as human albumin, branched-chain amino acids and reduced glutathione, depending on the child’s condition, to further treat hypercholesterolemia. In the case of congenital biliary atresia, children with hypercholesterolemia need to be seen in pediatric surgery for further management and to determine whether liver transplantation is needed.