Objective To discuss diagnosis and treatment strategy of vanishing bile duct syndrome. Method The clinical data of 1 child patient with suspected biliary atresia who received the liver transplantation in the West China Hospital of Sichuan University was retrospectively analyzed. Results The child patient underwent the liver transplantation due to the liver failure, and the postoperative pathological results indicated the vanishing bile duct syndrome accompanied by the formation of secondary (incomplete segmentation) cholestatic liver cirrhosis, the postoperative recovery was good. After 11 months of follow-up, the liver function, coagulation function, and blood flow of the transplanted liver were not obviously abnormal. The height and weight were suitable for the same age. Conclusion At present, there are many etiologies and complicated pathogenesis of vanishing bile duct syndrome, and liver transplantation is an ultimate effective treatment.