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. © 1993.