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Portal hypertension is a syndrome marked by an increase in the pressure of the portal vein. Portal hypertension can be diagnosed clinically or if the measurement of the hepatic venous pressure gradient is greater than 5 mm Hg. Cirrhosis is the most common etiology in Western countries, but there are other causes which lead to presinusoidal portal hypertension. We present a patient with a rare cause of portal hypertension.