A benign villous tumor of the second duodenum was treated by endoscopic excision. Fifty-four previously published cases are reviewed. Villous tumors of the duodenum are very uncommon. Presenting features include intestinal bleeding and/or occlusive symptoms and/or obstructive jaundice. Diagnosis is established by fiberoptic endoscopy and biopsies. Complete removal of the tumor is mandatory because of the high incidence of malignant changes (approximately 33%). Endoscopic excision is only possible in a small number of cases, when the tumor is small, benign, and pedunculated. Complete removal of the tumor ald unequivocal diagnosis of benign tumor on histological examination are required to withstand from further therapy. The best procedure is often surgical excision or segmentary duodenal resection. This is satisfactory for benign lesions or tumors with focal in situ malignant changes. Pancreatoduodenectomy should be considered for invasive malignant tumors.

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