The aim of this study is to present our institutional experience in living donor liver transplantation (LDLT) as a treatment for end-stage liver disease in children with biliary atresia (BA). A retrospective review of transplant records was performed. One hundred BA patients (52 males and 48 females) underwent LDLT. The mean follow-up period was 85.5 months. The mean age was 2.4 years. The mean preoperative weight, height, and computed GFR were 12.2 kg, 82.5 cm, and 116.4 ml/min/1.73 m 2 , respectively. Twenty-seven patients were below 1 year of age, and 49 patients were below 10 kg at the time of transplantation. Ninetysix had had previous Kasai operation prior to transplant. The mean recipient operative time was 628 min. The mean recipient intraoperative blood loss was 176 ml. Thirty-five did not require blood or blood component transfusion. The left lateral segment (64) was the most common type of graft used. There were 27 operative complications which included 3 reoperations for postoperative bleeding, 9 portal vein, 4 hepatic vein, 4 hepatic artery, and 7 biliary complications. There was one in-hospital mortality and one retransplantation. The overall rejection rate was 20%. The overall mortality rate was 3%. The 6-month, 1-year and 5-year actual recipient survival rates were 99%, 98% and 98%, respectively.