Alpha-1-antitrypsin deficiency is the most common inborn error of metabolism leading to liver transplantation, and the second cause of liver transplantation in children after biliary atresia. The authors report the case of a 6-year-old girl, who was suffering from end-stage liver disease secondary to alpha-1-antitrypsin deficiency. She was successfully treated by whole liver transplantation, the hepatic graft coming from a 3-year-old donor. Three months later she went back to school. The authors discuss the pathogenesis and the natural history of this frequent cause of liver transplantation in children.