Objective To investigate the effect of living donor liver transplantation plus domino auxiliary liver transplantation in the treatment of metabolic liver disease in children. Methods The first patient with ornithine aminotransferase deficiency（OTCD）received living donor liver transplantation（left lateral liver）and the patient's right lobe was procured for domino auxiliary donor liver transplantation. At the same time，the recipient of domino auxiliary liver transplantation was type Ⅰ crigler-najjar syndrome. The right half of the liver with the middle hepatic vein was resected，The domino liver was retained for vascular and biliary reconstruction. Results Recipie nt who received living donor liver transplantation had normal and sustained liver function and normal blood ammonia， the patient who received domino auxiliary liver transplantation had normal bilirubin and blood ammonia，Abdominal CT examination followed up one year after the transplantation was normal. Conclusion Non-sclerosing metabolic liver diseases can be treated by liver transplantation, and their livers can be used as domino donor livers，this type of liver graft can be successfully applied to auxiliary liver transplantation of different metabolic liver diseases，thus it provids new ideas for patients with metabolic liver diseases to expand the source of donor livers.