OBJECTIVE: To investigate the effect and prognosis of preserving the spleen and proximal splenic vein-portal vein shunt in the treatment of extrahepatic portal hypertension in pediatric patients. METHODS: From June 2011 to August 2013, four children, one male and three female, with an average age of 3.9 years, presented to our hospital with symptoms of upper gastrointestinal bleeding, including “vomiting blood and black stool”. The ultrasound and CT indicated portal spongiform changes, splenomegaly and hypersplenism, and the children were admitted with extrahepatic portal hypertension. All children were treated with extrahepatic portal hypertension by preserving the spleen and proximal splenic vein-portal vein left branch shunt. All children were followed up for 7-33 months (mean 20.8 months) after surgery. During the follow-up period, ultrasound was used to assess the spleen size and patency of the shunt vessels. Routine blood and blood biochemical test results were recorded. RESULTS: All children were successfully treated with a proximal splenic vein-portal vein left branch shunt. The operative time was 215-260 minutes, with an average of 237.5 minutes. 1 child received 1 unit of isotype red blood cells intraoperatively, and the remaining children did not receive blood transfusion. The postoperative hospital stay was 4-6 days, with a mean of 5.3 days. Postoperative portal venogram showed that all children had patent shunt vessels. Postoperative routine blood and biochemical results were normal, and follow-up ultrasound showed patency of the shunt vessels and significant reduction of the spleen. No child had recurrence. Conclusion: Proximal splenic vein-portal vein left branch shunt with preservation of the spleen is an effective treatment for extrahepatic portal hypertension in pediatric patients.