Diverse causes evoke Budd-Chiari syndrome including mallignant hepatic tumors such as hepatoblastoma inchildhood. The Budd-Chiari syndrome classically shows the symptom traid of ascites, hepatomegaly and abdominalpain. The radiologic findings include wall thinkening, stenosis, proximal dilatation of thrombosis of hepaticveins, hypertrophy of caudate lobe and collateral circulations on ultrasonography, ascites, hepatomegaly,nonvisualization or thrombosis of heptic veins, compression and flattening of inferior vena cava and collateralcirculations on CT, and definite hepatofugal flow from the liver on angiography. We have experienced a case ofBudd-Chiari syndrome associated with hepatoblastoma recently, so we report the case briefly with review.