A unique case of Paget’s disease of the anus is presented. The condition was entirely asymptomatic, produced no gross changes in the skin, and was associated with a polypoid adenocarcinoma of the anorectal junction. ThePaget cells persisted for more than a year after apparently complete removal of the tumor. Several histologic stains showed a close relationship between the tumor and the abnormal cells. Various concepts regarding the etiology of these mucus-producing cells are outlined, and an important reason for recognizing them in tissue sections is suggested.