Abstract: Congenital hepatic arterioportal fistula (cHAPF) is a specific vascular malformation and patients
with cHAPF do not present typical clinical manifestation. They can manifest as portal hypertension,
ascites and upper gastrointestinial bleeding. There is a great contrast between cHAPF and cirrhosis portal
hypertension in the etiology and pathologic physiologic course. Confirmation can be obtained by digital
subtraction angiography (DSA). Embolization plays an important role in the treatment of cHAPF. In this
paper, one case with cHAPF was reported and the involved literatures were reviewed and analyzed.