Budd-Chiari syndrome is an unusual form of portal hypertension, resulting from partial or total obstruction of the major hepatic veins or of inferior vena cava. This is illustrated by the clinical history of a two year old girl admitted by anemia, hepatomegaly, thrombocytopenia, pleural effusion, ascites and ultrasonographic, scintigraphic and angiographic evidence os suprahepatic veins and inferior vena cava obstruction. Liver biopsy showed signs of chronic congestion and fibrosis. Initial manifestation of illness may be acute or, as in this patient, with an insidious onset of symptoms. Medical therapy gives usually poor results. Several surgical approaches have been tried to alleviate liver congestion, which in this case was intended by a 6 mm wide Goretex ringed tube prosthesis extending from superior mesenteric vein throughout right atrium. Patency of the artificial shunt was proven after several days by Doppler ultrasound but the patient died of pneumonia two months after operation.