[Agenesis of the right lobe of the liver and portal hypertension].

A case of agenesis of the right lobe of the liver associated with portal hypertension is reported. This uncommon feature was diagnosed by computed tomography which excluded secondary liver atrophy due to cirrhosis or tumour. The main pathophysiological hypothesis for agenesis and portal hypertension, exceptionally associated, is arrest of hepatic development during foetal life.