CBA is the most common indication for liver transplantation in children, accounting for about 50% of all pediatric liver transplants, and in some larger pediatric liver transplant centers, it can account for more than 60% of all liver transplant cases. The etiology of this disease remains unclear, but it is generally believed to be related to intrauterine viral infection, inflammation of the small intrahepatic bile ducts secondary to obstruction, and congenital biliary developmental malformations. The intrahepatic bile ducts, common hepatic duct or common bile duct may be atretic or rudimentary, and the gallbladder may be atretic or hypoplastic. 25-30% of infants with CBA have other malformations in combination, such as circumscribed pancreas and congenital heart disease. Most of the infants have yellow stools for a week after birth, which gradually turn white after 1-2 weeks. This indicates that atresia of the biliary tract should occur after fetal bile production. In addition, biliary atresia has not been found in stillborn or preterm infants. None of these evidences support the doctrine of its congenital malformation and mostly favor the etiologic theory of inflammation. According to the site of extrahepatic biliary atresia, there are three types of biliary atresia: choledochal atresia, common hepatic duct atresia, and biliary atresia of the hepatic portal region. The incidence of this disease is about 1/20,000 in Europe and the United States and 1/10,000 in Japan, and it is higher in Orientals than in white Europeans and Americans. CBA is the most common cause of neonatal obstructive jaundice. The most common symptom is persistent jaundice following physiologic neonatal jaundice. Jaundice usually appears 1-2 weeks after birth or as late as 3-4 weeks. At the beginning of 1-2 months, the infant’s development is still normal, then developmental delay or lesions gradually aggravated, with the deepening of jaundice, the appearance of tea-colored urine and clay stools, severe cases due to the epithelial cells of the intestinal mucosa can exude bilirubin, which will make the grayish-white appearance of feces stained with light yellow. In the late stage, tears and saliva are also obviously yellow. The liver is markedly enlarged up to the umbilicus and beyond the mid-abdominal line. The spleen is enlarged in almost all cases. Abdominal distension, ascites and abdominal varicose veins may be present. Without effective treatment, most of the children die of liver failure in about 6 months. In the early stages of the disease, the children have a good appetite and nutritional status; in the late stages of the disease, due to impaired absorption of fat and fat-soluble vitamins, they become progressively weaker, and vitamin A, D, and K deficiencies may occur, resulting in dry eye, rickets, and hemorrhagic tendencies. Ultrasound examination reveals an enlarged liver with no normal right or left hepatic ducts or common bile ducts, and the gallbladder is often undetectable or atrophic, although biliary atresia cannot be excluded if the gallbladder is detected. If the gallbladder is detected, biliary atresia cannot be completely excluded, because intraoperative exploration of the gallbladder is often grayish-white transparent fluid rather than bile. Serum direct bilirubin and alkaline phosphatase are persistently elevated on laboratory tests. Liver function is normal in the early stages with abnormal changes in the late stages. Urine bilirubin is positive and urine bile element is negative. Nuclide 99mTc-IDA (acetanilide diacetate) imaging is available, and the absence of radio-concentration in the gut within 6 hours of tracer sedation supports the diagnosis of the disease. Almost all patients with CBA died before the 1960s, but in the last 30 years, Kasai, a Japanese scholar, has proposed hepatic-portal hepatic-intestinal anastomosis (i.e., Kasai’s procedure), which gives hope for the survival of children who were “inoperable” in the past. For children with biliary atresia, once the diagnosis is clear, the Kasai operation is usually performed within 2 months, when the success rate of the operation is high, and the operation after 4 months of life is not effective. Among them, 20% to 30% can have normal growth and development after surgery; 1/3 of the children can have their symptoms relieved, and their serum bilirubin can be gradually reduced to normal level, but the cirrhosis and portal hypertension caused by recurrent cholangitis after surgery will affect the long-term efficacy of the operation; some of the children, even if effective bile drainage is established, cannot stop the process of hepatic fibrosis, and the mortality rate of the children within 5 years is 90%. Most liver transplant centers now recommend Kasai for children with CBA. Pediatric liver transplantation provides an effective treatment and is the only life-saving procedure for infants who do not improve after Kasai or who survive after too late treatment. Relative living donor liver transplantation is the ideal procedure for this group of patients, solves well the problem of source and matching of donor liver for pediatric liver transplantation, and has good graft results. It is usually better to perform the procedure when the patient is older than 1 year old and weighs more than 10 kg. If children with recurrent cholangitis, cirrhosis and portal hypertension appear after Kasai operation, transplantation should be performed as early as possible at the age of 2 to 5 years, otherwise the efficacy of liver transplantation will be affected when the systemic condition is too poor, and the survival rate of liver transplantation can be up to 68-95% at 5 years after the operation.Srinivasan et al. reported that the patients with CBA were often combined with the deformity of vena cava, which made it difficult to anastomose the vessels in transplantation, and there were more complications after the operation. Srinivasan et al. reported that patients with CBA often have malformation of the vena cava, which makes vascular anastomosis difficult during transplantation and causes many postoperative complications, and that reconstruction of the inferior vena cava using iliac vessels can achieve better transplantation results.