Byler’s disease (BD) is a heterogeneous group of autosomal recessive childhood cholestatic diseases of hepatocellular origin. It has a rare incidence of l in 90000. It is characterized by cholestasis often arising in the neonatal period and it leads to death, due to liver failure, without liver transplantation (OLTx). Publishes data on survival and post OLTx complications are limited. From October 1997 through January 2004, 242 pediatric OLTx were performed in 215 patients (pts) at “Ospedali Riuniti di Bergamo”. BD was the indication in 12 pts, with age ranging from 0.7 to 13 years (median 1.32). We reviewed the data of these pts for technical features of the OLTx, incidence and type of complications, need for re-transplantation and patient and graft survival. There were 8 males and 4 females with a median weight of 10 kg (6-35). 7 Pts (58.3%) received a LLS graft (one of these was a reduced LLS graft), 5 pts (41.6%) received a whole graft. Median follow up was 670 days (3-2051). During the same period no pts died while on the waiting list. Major surgical complication requiring a reintervention occurred in 3 pts (25%) (Bowel perforation, skin flap for necrotic lesions of the abdominal wall, portal vein thrombosis). No thrombosis of the hepatic artery was observed. Infections with positive blood cultures were diagnosed in 4 pts (33.3%). One patient (8.3%) had biliary anastomotic stenosis successfully treated by percutaneous techniques. Four pts (33.3%) had an episode of acute rejection that was successfully treated with steroids. Two pts (16.6%) were retransplanted, one for portal and caval thrombosis with a LLS graft and the other for primary dysfunction with a right extended split graft. Both of them died in the early post operative period after reOLTx, respectively for sovra-hepatic vein thrombosis and for veno-enteric fistula. All the other pts are currently alive with an actuarial pt and graft survival of 83% at 1 yr and 83% at 5 yrs. Our results confirm that Tx is an effective treatment for children with BD. Split liver technique satisfies the need for grafts for these pts.