- background. Basal cell carcinoma (BCC) of the nipple is a very unusual occurence, often mistaken for Paget's disease. objective. By reporting a further case of BCC of the nipple in a 57-year-old man and by reviewing the sixteen previously reported cases, we mean to characterize further a common lesion arising at an unusual site. We also wish to stress the lack of aggreement regarding the treatment of BCC of the nipple. methods. Review of the literature was based on a search in Medline. conclusions. We appeal to compile all cases of BCC arising at unusual locations in order to gain a better understanding of these lesions.