Benign tumors of the papilla of Vater are unusual; fewer than 100 cases have been reported. Six cases with adenomyosis of the papilla of Vater are presented. In two patients, the adenomyosis was an incidental finding at laparotomy or autopsy. In two patients, adenomyosis produced symptoms, and the lesions were successfully excised. There was no recurrence of biliary symptoms postoperatively. In two patients adenomyosis was associated with adenocarcinoma of the papilla of Vater. Adenomyosis can be a premalignant condition. Frozen section for histologic examination should be done when a tumor is encountered in the papilla of Vater. Adequate local excision of a benign tumor should result in cure. The patient must, however, be followed-up carefully, and a reexploration should be performed if there is the slightest indication of recurrence. The recurrence is often malignant.