Occlusive PVT concurrent with chronic liver disease is a common clinical entity among pediatric patients referred for transplantation. The natural history of PVT is unknown. Our aim was to determine, using a retrospective cohort design, if children under 13 yr with chronic liver disease and concomitant PVT have an increased mortality risk prior to and after transplantation. A total of 203 patients were included in the study. Nearly 10% of the population had PVT (n = 19); 63.2% of PVT patients (5.9% of total cohort) underwent liver transplantation (n = 12). PVT patients tended to be younger than non-PVT patients at evaluation (1.94 +/- 3.51 vs. 3.79 +/- 4.11, p = 0.059). Clinical and demographic factors were similar between the two groups. Regarding survival, four PVT patients died, of which two had undergone transplantation. Kaplan-Meier analyses indicated that PVT and non-PVT patients had similar survival from the time of evaluation, on the waiting list, and after transplant. Although limited by sample size, our study suggests that a diagnosis of PVT does not increase the mortality risk for children waiting for a liver transplant. Further study is needed to discern variations in mortality risk that may occur in the pediatric chronic liver disease population with PVT.