A common tumor, an uncommon location: basal cell carcinoma of the nipple and areola in a 49-year-old woman.

Background: Basal cell carcinoma (BCC) occurring on sun-protected regions is an uncommon phenomenon. BCC of the nipple is an exceedingly rare event.

Method: We review the literature on BCC of the female nipple and herein describe the eighth reported case in the English literature. Our patient was treated with Mohs micrographic surgery and sentinel lymph node biopsy.

Conclusion: BCC of the nipple are extremely rare tumors with unclear etiology. They can be aggressive and are capable of causing significant morbidity and mortality if they are neglected or improperly treated. With continued reporting of the diagnosis, treatment, and follow-up of these patients, we may gain an understanding of the pathogenesis, as well as the best method of control for these unusual tumors.