Congenital biliary atresia is a common cause of obstructive jaundice during the neonatal period. Moreover, early biliary atresia is extremely difficult to distinguish from neonatal hepatitis syndrome, especially when neonatal hepatitis is in the obstructive phase. Therefore, early differential diagnosis should be made, and once biliary atresia is diagnosed, surgery should be sought within 40-60 days after birth. Duodenal-jejunal anastomosis should be done for the possible anastomosis type of hepatic duct and common bile duct atresia, and hepatoportal-jejunal anastomosis for the impossible anastomosis type; for those whose disease duration is close to two months and the diagnosis is still unclear, surgical exploration can be done. For children within 90 days, hepatic hilar-jejunostomy should be pursued, and liver transplantation can be done if the surgery fails. For children older than 90 days, conditions can be created for liver transplantation. Hepatic hilar-jejunostomy is currently recognized as a procedure with therapeutic value for biliary atresia, but the long-term outcome does not reach a satisfactory level. The timing of the procedure and the surgical technique are important factors affecting the prognosis. The postoperative bile drainage, the frequency and degree of postoperative cholangitis, and the degree of damage to the liver parenchyma can also affect the prognosis. Liver transplantation may be considered for those who have failed hepatic hilar-jejunostomy treatment or are too old and available.