Background:As an uncommon cause of portal hypertension, splenic arteriovenous fistula (SAVF) may show as stomach discomfort, diarrhea, ascites, and/or hematemesis. Formation of a fistula may be traumatic or spontaneous..[1,2] Case Presentation : A 7 years old Female with history of recurrent episodes of hematemesis, endoscopy in 2021 showed tortous esophageal varices with multiple site of bleeding, Thoraco-abdominal CTA in 2021 showed thrombus in portal vein and Splenomegaly. During operation there was incidental finding of SAVF, the fistula then ligated and followed by retrograde thrombectomy portal vein and distal splenorenal shunt. Post operative abdominal ultrasound showed portal vein diameter 6mm with velocity 15.7cm/s, there was no recurrent hematemesis and patient was discharge on 7th day post operation