Congenital intrahepatic arterioportal fistula (IAPF) is a rare condition and there is limited experience of transcatheter embolization. We report here the transcatheter coil embolization of an incidentally found, asymptomatic congenital IAPF in a 16-month-old patient. After demonstrating that the IAPF was fed by two branches of a dilated left hepatic artery and drained into an aneurysm of the left portal vein, the feeding arteries were superselectively catheterized and occluded by microcoils in a single session. Follow-up was uneventful for 1 year. With respect to this and previously reported cases, we believe that embolization of feeding arteries is a safe and effective method that should be the primary choice of treatment; however, further cases should be reported to confirm this belief.
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