Hepatocellular carcinoma and other tumours of the liver are extremely rare in Wilson's disease. We report a patient who presented with a cholangiocarcinoma associated with Wilson's disease. The literature review underlines that patients with Wilson's disease should be considered at risk of hepatocellular carcinoma, cholangiocarcinoma and undifferentiated carcinoma in the liver. Risk factors seem to be long disease duration and probably a poor observance to therapy. A liver imaging should be included in the follow-up of patients with Wilson's disease.
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