A case of portal hypertension in a five-year-old with multiple hydatid cysts in the liver is reported. Compression of the portal vein was the likely mechanism. Abdominal ultrasonography disclosed four hydatid cysts of which the largest was located in segments VII and VIII and caused compression of the supra-hepatic veins and inferior vena cava. Doppler ultrasonography showed continuous venous flow without triphasic modulation in the middle suprahepatic vein and inferior vena cava. Abdominal computed tomography demonstrated compression of the inferior vena cava and failed to visualize the suprahepatic veins. Upon surgery, evidence of hepatic venous statis was found. The child died 24 hours post-surgery. Budd-Chiari syndrome should be looked for routinely in patients with hydatid disease of the liver.
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