Ninety three out of 96 patients with biliary atresia (BA) underwent Kasai 1 portoenterostomy and 11 children subsequently underwent 12 liver transplantation (LTX) procedures which included 8 living related liver transplants (LRLT), 3 reduced-size liver transplants (RSLT) and 1 orthotopic liver transplantation (OLT). During a follow-up period of 3-206 months (mean, 73 months) after portoenterostomy and 3-63 months after LTX, 50% of 96 patients are alive and well with total clearance of jaundice while 12% are mildly jaundiced, 10% are deeply jaundiced and 28% have died. Two of the 3 patients who did not undergo portoenterostomy and 25 from the portoenterostomy group have died. Of the latter group, 22 deaths occurred before, and 3 after the introduction of LTX therapy respectively. Of the 68 long-term survivors, 32 are less than 5, and 36 are 6-17 years old. Complete clearance of jaundice was achieved in 55% of patients irrespective of whether portoenterostomy was initially performed at < 10 or between 10 and 12 weeks. Portoenterostomy performed beyond 13 weeks was associated with declining results. We conclude that (1) portoenterostomy combined with liver transplantation, when indicated, has given patients with BA a much better prognosis and (2) pediatric LTX in our institute is a well established procedure with 100% patient and 88% primary graft survival.