Biliary Atresia (BA) has an incidence of approximately one in 10000 live birth worlwide. Standard surgical treatment is represented by hepatoporto-enterostomy (Kasai procedure) but failure of this operation and subsequent need for Liver Transplantation (OLTx) occurs in as much as 75% of the patients (pts) representing more than 50% of the pediatric candidates to OLTx itself. However published data on survival and post OLTx complications in large series and recent years are limited. From October 1997 through December 2003, 240 pediatric OLTx were performed in 213 patients (Pts) at “Ospedali Riuniti di Bergamo”. BA was the indication for OLTx in 129 pts, with a median age of 0.9 years (0.2-16.7). We reviewed the data of these pts for technical features of the OLTx, incidence and type of complications, need for reOLTx, pt and graft survival. There were 65 males 64 females, with a median weight of 9 kg (4-62) 102 pts (79%) were younger than 2 yrs. 99 Pts (76.7%) received a LLS graft (4 reduced LLS graft), 24 pts (18.6%) received a whole graft, 4 (3.1%) received a I+IV-VIII segment, and 2 Pts (1.5%) received a I-IV segment. Mean follow up was 1050 days (94-2293). During the same period no pt died on the waiting list. Infections with positive blood cultures were diagnosed in 45 pts (34.8%). Surgical complications that required reintervention occurred in 22 pts (17%) (9 bowel perforations, 8 eviscerations, 5 hemoperitoneum). 12 pts (9.3%) had hepatic artery thrombosis. 29 pts had biliary complications (22.4%), 17 of them were successfully treated by percutaneous technique, whereas 12 needed surgical revision. 14 (10.8%) pts were retransplanted, 8 for arterial thrombosis, 3 for PDF, 1 for portal thrombosis, 1 for chronic rejection, 1 for SE Veins thrombosis, all received a LLS graft. In two cases another re OLTx was performed for arterial thrombosis, these pts received a LLS graft too. 40 pts (31%) had acute rejection treated with steroids. Currently 116 pts are alive, with an actuarial survival at 1 yr of 92% and 91% at 5 years; the graft actuarial survival is 85% at 1 yr and 82% at 3 and 5 yr. The actuarial survival of the pts that receiving LLS graft (split in situ and ex situ) is 93.6% at 1 year and 92% at 5 years, the graft actuarial survival, of these pts, is 87.3% at 1 year and 85.6% at 5 years. To the best of our knowledge this is the largest published series of OLTx for BA all performed in the last decade. Our results confirm the effectiveness of OLTx in treatment of children with BA and a failed Kasai procedure. Split liver grafts represent an excellent organ supply for these patients; allowing achieving optimal results with no mortality on the waiting list. Cholestatic diseases.