Basal cell carcinoma arising from the nipple of the breast is an uncommon malignancy, with only a handful of cases reported in the literature. The diagnosis is, however, important because basal cell carcinoma in this location can mimic breast cancer, may behave aggressively, and there is a relative lack of consensus in its treatment. A case of basal cell carcinoma of the nipple is described in a 67-year-old man with a brief review of the literature.