Diverse causes evoke Budd-Chiari syndrome including malignant hepatic tumors such as hepatoblastoma in childhood. the Budd-Chiari syndrome classically shows the symptom triad of ascites hepatomegaly and abdominal pain. The radiologic findings include wall thinkening stenosis proximal dilatation of thrombosis of hepatic veins hypertrophy of caudate lobe and collateral circulations on ultrasonography ascites hepatomegaly nonvisualiza-tion or thrombosis of hepatic veins compression and flattening of inferior vena cava and collateral circulations on computed tomography and definite hepatofugal flow from the liver on angiography. We have experienced a cases of Budd-Chiari syndrome associated with hepatoblastoma recently so we report the case briefly with review.