Increasing numbers of children undergo successful liver transplantation. Limited data exist on long-term survival and late graft loss. Survival and graft loss were studied in 376 primary liver graft recipients who survived more than 3 months after transplantation (80.5% of all primary graft recipients). Patient records were reviewed retrospectively for causes of graft loss. Risk factors were identified by analyzing graft, recipient, and posttransplant variables using multivariate Cox regression. One-, 5-, and 10-year actuarial graft survival rates in the study population were 94.6%, 87.3%, and 86.3%, respectively. Corresponding patient survival rates were 95.7%, 91.4%, and 90.4%. Forty-seven (12.5%) grafts were lost subsequently, 15 by patient death with preserved graft function. Survival rate after late retransplantation was 63.3%. Causes of late graft loss were infection (21.2%), posttransplant lymphoproliferative disease (PTLD, 21.2%), chronic rejection (17%), biliary complications (14.8%), and recurrence of malignant disease (8.5%). Independent risk factors for late graft loss and patient death included liver malignancy as primary disease, steroid resistant rejection, and PTLD. Graft loss rate was significantly increased for reduced-size grafts. Patients undergoing transplantation after 1991 and recipients of full-size grafts were more likely to survive. In conclusion, the long-term outcome for pediatric primary liver graft recipients surviving the early postoperative period is excellent except for patients with liver malignancy. There is no increased risk of late graft loss with the use of split or living related donor grafts. Technical complications are only a minor factor in late graft loss, but complications related to immunosuppression and infection remain a major hazard and must be addressed. (Liver TranspZ2002;8:615-622.) 0 rthotopic liver transplantation (OLT) is considered standard therapy for children with end stage liver disease, fulminant hepatic failure, and selected metabolic diseases, e.g., hyperoxaluria type 11. Patient survival after OLT approaches 90% at 1 year, 85% at 5 years, and over 75% at 10 years posttransplant in experienced centers.'p2 Nevertheless about 20% of primary grafts are still failing, the majority in the early postoperative period. Much attention has been focused on early graft loss, but with improving long-term survival the risk of late graft loss is becoming an important issue. Late mortality and its causes after pediatric OLT have been analyzed,3 but there are no large-scale data concerning incidence, causes, and risk factors of late graft loss. Results of studies in adult patients are not applicable to children because of the different spectrum of diseases leading to OLT in adults. Identification of risk factors for late graft loss may help in reducing the frequency of retransplantation and, hence, the risk of patient death, which is significantly increased after retransplantation.* Furthermore, in an era of donor organ shortage and increasing financial constraints, it...