Although basal cell carcinoma (BCC) of the nipple-areola complex (NAC) is rare, it is important for dermatologists to be aware of this potential malignancy, as it is thought to behave more aggressively than BCC arising in other anatomic locations and also can mimic a number of more serious conditions. A review of the literature failed to generate a consensus regarding staging or treatment of BCC of the NAC; current therapies range from simple excision of the lesion to mastectomy with sentinel lymph node biopsy. We report the case of a 23-year-old man who presented with a pigmented BCC of the nipple to highlight several important aspects of the diagnosis; we also review 49 cases of BCC of the NAC from the literature and give our recommendations for treatment approach.