There are various causes of cholestatic liver disease in infants that include extrahepatic biliary tract diseases, liver diseases, anatomical abnormality, metabolic or endocrine diseases, poisoning, infection, and so on. The causes leads to re-duction or interruption in bile lfow and thus results in nutrients malabsorption and comprehensive metabolic disorder. The aims of nutritional therapy for cholestatic liver disease are to reduce the risk of cholestasis related complications, to promote recov-ery of liver diseases, to restore bile flow, to promote growth and improve the comprehensive metabolic disorders, to improve quality of life and to prepare children for liver transplantation.