An intrahepatic arterial-portal venous malformation (APVM) was diagnosed in a 4 1/2-month-old infant with portal hypertension. Visceral angiography showed a large saccular vascular space in the left hepatic lobe with a multiplicity of feeding arteries and drainage into the left portal vein. The lesion was successfully treated by transcatheter embolization following two unsuccessful surgical procedures. Two years later portal vein thrombosis with cavernous transformation was diagnosed, possibly a consequence of the high-flow arterial-portal shunt and its subsequent occlusion. Knowledge of the anatomic differences between this rare congenital APVM and the more commonly occurring arterial-portal fistula is crucial in planning effective transcatheter embolotherapy or surgery for these lesions.