Biliary atresia (BA) is a major cause of extrahepatic biliary obstruction in children. Malnutrition is a significant clinical problem in children with BA. BA may induce the malabsorption of fat and fat-soluble vitamins, resulting in cholestasis and an impaired nutritional status. For the treatment of BA, it is most important to reconstruct the bile flow as early as possible by performing a Kasai portoenterostomy. After the Kasai operation, growth and nutrition are restored, but to follow normal growth and development, it is necessary to evaluate the nutritional status and support. Therefore, the purpose of nutritional support in children with BA is to normalize growth and development, prevent further liver damage and deterioration of the patient’s nutritional status, avoid vitamin and mineral deficiencies, and improve the quality of life of patients.