Cavernous transformation of portal vein (CTPV) is the main cause of portal hypertension and its related variceal bleeding in children. Transjugular intrahepatic portosystemic shunt (TIPS) was not reported to treat CTPV for children younger than 5 years old. In this case report, the patient was a 26-month-old boy who presented with hematemesis and melena due to CTPV. Even after azygoportal disconnection, repeated hematemesis as well-melena still occurred. After careful evaluation, we performed TIPS under general anesthesia for him. The procedure was uneventful, and 6-mm stents were inserted. Six months after TIPS, there was no recurrence of bleeding, and no procedure-related event happened. The follow-up esophagogastroduodenoscopy proved dramatic remission of varices, indicating a successful outcome. We believe TIPS could be safely placed for young children to manage variceal bleeding due to CTPV.