Abstract: AbstractObjective：To describe the imaging features of congenital portosystemic shunt presenting in 8 children with gastrointestinal bleeding.Methods：This retrospective, singlecenter study was approved by our institutional review board. We searched the cases of congenital portosystemic shunt with history of gastrointestinal bleeding in hospital information system of Children's Hospital of Fudan University. All patients' medical records including sex, age, clinical manifestations, CTA and MRA imaging data, and surgical outcome were reviewed. The raw CT data were reconstructed into 1.25mm slice thickness and 0.625mm slice interval, and then were sent into a CT workstation for the postprocessing. Also, the raw MR date were sent into an MR workstation for postprocessing. After the data were postprocessed, all of the images were sent to the Picture Archiving and Communication System and were reviewed by 2 radiologists with more than 10 years experience in pediatric abdominal CT and MR imaging; both radiologists were blinded to the clinical data and identified the main portal vein, intrahepatic portal vein, internal iliac vein and superior rectal vein, as well as the portosystemic shunts. A consensus interpretation of image findings between them was accepted.Results：A total of 8 consecutive cases with the inclusion criteria were included in this study from March 2008 to January 2017. 6 were male, 2 were female, age was ranged from 3 months to10 years. 7 patients presented with lower gastrointestinal bleeding, 1 with hematochezia, 7 patients with anemia. All patients underwent CTA examination, 2 of them underwent MRA examination at the same time. By the time of discharge, 6 cases received surgical treatment, and 2 cases were followed up. All of the 8 cases were congenital extrahepatic portosystemic shunt, 6 were type Abernethy Ⅱ and 2 were type Abernethy Ⅰ. The images of the seven patients with hematochezia showed that the shunts were from the splenomesenteric junction (n=6) or splenic vein (n=1) to the internal iliac vein via an inferior mesenteric vein .One patient with hematemesis showed the shunt from the splenomesenteric junction to the left renal vein via the venae coronaria ventriculi.Conclusion：Most of congenital portosystemic shunt patients with gastrointestinal bleeding had a shunt that drained portal blood into the iliac vein via an inferior mesenteric vein. This type of shunt was not common in congenital portosystemic shunt, but the prevalence of gastrointestinal bleeding in patients with this type was high.