Objective To analyze the incidence and risk factors of portal vein stenosis (PVS) in pediatric liver transplantation (LT). Methods This retrospective analysis of 396 cases of pediatric LT (patients aged <= 14 years old) was conducted at the Liver Transplantation Center of Beijing Friendship Hospital (China) from June 2013 to December 2017. We collected relevant data and calculated the incidence. We analyzed a total of 23 risk factors for PVS children during the perioperative period. Results The incidence of PVS in pediatric LT was 6.6%. The following were identified as risk factors for PVS in pediatric LT: Preoperative portal hypertension was complicated, weight (<= 7 kg), recipients of portal vein diameter <= 4 mm, GRWR (>= 3.5%), the use of cold preservation vein grafts, anastomosis in the region of superior mesenteric vein and splenic vein and reverse blood flow in the portal vein shown in preoperative ultrasound examination. Recipients of portal vein diameter <= 4 mm and the use cold preservation grafts were independent risks factors for PVS in pediatric LT. Conclusion For recipients with the risk factors identified in this study, we strongly recommend a strict follow-up and the provision of suitable interventions when indicated.