A 63-year-old man with genetic haemochromatosis underwent resection of a cholangiocarcinoma that developed in von Meyenburg complexes; the liver was not cirrhotic. Patients with an association of genetic haemochromatosis and von Meyenburg complexes might have a predisposition to cholangiocarcinoma, even before cirrhosis occurs. Patients with this association should undergo regular and early hepatic surveillance of hemochromatosis.