Congenital agenesis of the right lobe of the liver is a rare anomaly. In a case report the diagnosis was made by computed tomography. A retro- or suprahepatic location of the gallbladder and compensatory hypertrophy of the left liver lobe and/or caudate lobe may give support to the diagnosis. Before diagnosing congenital agenesis of the lobe, lobar atrophy due to cirrhosis, cholangiocarcinoma or any other predisposing conditions should be eliminated.
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