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A patient with obstructive jaundice was found to have bile duct carcinoma and right hepatic lobe agenesis. The diagnosis was made by computed tomography (CT), cholangiography, and angiography. Right hepatic lobe agenesis is a rare anomaly and has never been previously reported with bile duct carcinoma. CT cholangiography was critical in diagnosing the morphologic anomalies of the bile duct in this case.
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