BACKGROUND: Biliary atresia can be treated by portoenterostomy, which is primarily palliative, or by liver transplantation, which is primarily curative. The purpose of this study was to determine the long-term outcome of liver transplantation for the treatment of biliary atresia. METHODS: During an 8-year period, 45 patients who underwent liver transplantation for biliary atresia and 10 patients who were referred to our center for portoenterostomy were retrospectively analyzed. RESULTS: No patient with biliary atresia died awaiting liver transplantation. The waiting time for all patients was 36.7 +/- 42.8 days. Thirty-four patients (75.6%) required one transplant, whereas 11 patients (24.4%) required 17 retransplants. Twenty-two patients (48.9%) required 39 reoperations (1.8 per patient). There were 4.9 infectious episodes, 2.2 rejection episodes, and 4.4 readmissions per patient. However, 91% of reoperations, 80% of infections, and 78% of rejections occurred within 6 months of transplantation. The overall 7-year actuarial patient and graft survival for patients with biliary atresia was 86.2% and 62.7%, respectively. CONCLUSIONS: Our results indicate that long-term patient survival after liver transplantation for biliary atresia is excellent. However, portoenterostomy continues to have an initial complementary but limited long-term role in the treatment of infants with biliary atresia.