Congenital hepatic arterioportal fistula (HAVF) is extremely rare in children. We present a patient with congenital hepaticoportal arteriovenous fistula complicated with gastrointestinal bleeding treated using transcatheter arterial embolization. Our patient was the youngest (2 days old) case ever reported with congenital HAVF and the first one to receive arterial embolization for HAVF during childhood. The 3-year-old girl was suggested of having congenital HAVF using Doppler ultrasonography. However, her family refused further investigation, and she was lost to follow-up. Three years later, she was sent to our hospital due to melaena. Repeated ultrasonography revealed dilated intrahepatic portal vein with arterial flow demonstrated using Doppler imaging. No esophageal varices or gastric or duodenal ulcer was seen during endoscopy. Angiography showed a HAVF and transcatheter embolization was done simultaneously. Follow-up at one and two weeks post-embolization revealed no more shunt flow within the portal vein, though cystic like dilatation of the portal vein persisted, and no thrombosis was observed. This case emphasizes that transcatheter arterial embolization can be easily and successfully used for treating childhood congenital HAVF. Abnormal dilatation of the portal vein in children needs doppler evaluation and possibly angiography.