Biliary atresia is the most common cause of chronic cholestasis in infants and children. The incidence is estimated at 3.7:10,000 among Taiwanese infants. Kasai hepatoportoenterostomy helps children survive beyond infancy. Liver transplantation is indicated when the Kasai procedure fails to work or when patients develop progressive deterioration of liver function despite an initially successful Kasai operation. Living donor liver transplantation was developed to alleviate organ shortage from deceased donors. It has decreased the waiting time for transplantation and, therefore, improves patient survival. One hundred living donor liver transplantations have been performed for biliary atresia at Chang Gung Memorial Hospital-Kaohsiung Medical Center with both 98% 1-year and 5-year actual recipient survival.