Introduction: Biliary complications (BC) still remains the ‘Achilles heel’ of pediatric liver transplantation (LT). The aim of the study was to describe the incidence of BC and analyze the impact of surgical treatment of BC on long-term patient and graft survival. Methods: We retrospectively reviewed 429 primary LT performed at St. Luc University Clinics, Brussels, Belgium, between 01-07-1993 and 01-12-2010. The median recipient age at LT was 1.6 years (range: 0.2 - 17.5). The main indications for LT were primary biliary atresia (58%) and progressive familial intra-hepatic cholestasis (8%). 21 (n=88) recipients received a whole liver graft. The incidence of technical variant techniques namely reduced liver, split liver and live-donor was 21% (n=91), 11% (n=47) and 73% (n=203), respectively. The median follow-up was 7.6 years (range: 6 mo - 17.6 yrs). Results: The overall 1, 5, and 10-year patient and graft survival rates were 98%, 95% and 94%, and 97%, 94% and 92%, respectively. At 5 years, the overall incidence of BC was 23% (n=98). 60 of them were anastomotic complications (47 (78%) strictures and 13 (22%) fistulae), all of them, except one, primarily surgically treated. Surgical treatment of biliary anastomotic strictures consisted in resection of the stenotic bile duct tissue followed by a reconstruction of the biliary anastomosis. The type of the graft was not found as an independent risk factor for the development of BC. At multivariate analysis, only acute rejection and hepatic artery thrombosis increased the risk of BC (Odds ratio 1.76 (1.06 - 2.92);p=0.03 and Odds ratio 3.07 (1.18 - 8.05);p=0.02, respectively). The success rate of the surgically treated BC (defined as the normalization of liver enzymes, the disappearance of dilated bile ducts on ultrasound, absence of repeated biliary surgery and the absence of impact on graft and patient survival) was 80% (n=48). The 1, 5 and 10-year patient and graft survival rates of surgically treated BC were comparable with recipients without BC (p=0.553 and p=0.398, respectively). Conclusion: Despite the excellent outcome of pediatric LT, BC still represent a major source of morbidity, the majority being anastomotic complications. Our results suggest that surgical management of anastomotic BC may constitute the best and the first therapeutic option in terms of subsequent patient/graft survival. The respective role of surgery and interventional radiology for recurrent biliary anastomotic strictures requires further analysis.