Summary: Background: Authors are presenting a case of papillomatosis of the common bile duct, causing obstructive jaundice, which is a rare diagnosis. Methods: On the base of the preoperative examinations and the operative finding an early malignant tumor of the common bile duct was suspected, and only the postoperative pathohistology examination verified the proper diagnosis. Results: The patient treated with pancreatoduodenectomy is healthy two years after the operation without any complaints. Conclusions: The authors emphasize that the radical operation is mandatory in the case of this benign disease as it has an inclination for recurrence or malignant transformation.