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Mucinous cholangiocarcinoma, characterized by large quantities of mucin production, is a rare subtype of peripheral cholangiocarcinoma and usually shows rapid progression and a fatal outcome. We report here a case of mucinous cholangiocarcinom a in a 69 year-old man, who was infected with Clonorchis sinensis. Histologically, the tumor was an adenocarcinom a with extensive intracellular and extracellular mucin production, up to 70% of the tumor mass and there was frequent lymphovascular invasion of the tumor cells. The liver adjacent to the mass contained eggs of C lonorchis sinensis in the bile duct lumen and showed ductal epithelial hyperplasia, mucinous metaplasia and adenomatous proliferation of intramural glands. The patient was treated with a right hepatectomy. Four months after the surgery, the tumor recurred in the soft tissue of the right flank. (Korean J Hepatol 2004;10:223-227)