This study was designed to evaluate the validity of the pediatric end-stage liver disease (PELD) score as a prognostic index of native liver survival in biliary atresia before Kasai portoenterostomy. Medical records of 63 patients who underwent Kasai portoenterostomy at our hospital were reviewed retrospectively. Rate of jaundice clearance and cumulative survival with native liver were calculated. Patients who were alive and did not require transplantation until the last follow up were included in the native-liver survival group. And patients who underwent any event, defined as death without transplantation, transplantation or listing for transplantation, were included in the native-liver failure group. Cox analysis was done. 10-year survival rate with native liver was 41.9%. PELD scores of ≥ 15 and an age at operation of ≥ 60 days were found to be significant risk factors of failure(P = 0.007, OR = 2.851 and P = 0.010, OR = 3.222, respectively). Furthermore, patients with both risk factors showed significantly higher risk of failure (P = 0.001, OR = 8.809). Since failure rate is higher for patients with high PELD score and older age, these factors should be helpful in estimating the prognosis. And with high-risk patients, cautious monitoring and consultation should be made whether the liver fails and requires transplantation.