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 m2, respectively. Twenty-seven patients were below 1 year of age, and 49 patients were below 10 kg at the time of transplantation. Ninety-six 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.