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Basal cell carcinoma (BCC) occurring on sun-protected regions is an uncommon phenomenon. BCC of the nipple is an exceedingly rare event, often mistaken for Paget's disease. BCC is the most common human malignancy of the skin cancer, accounting for approximately 75% of all nonmelanoma skin cancers diagnosed in the United States. Most BCCs are sporadic and occur on the sun-exposed areas of the head and neck. Rarely, these tumors have been reported to develop in non-sun-exposed areas, such as the axilla, perineum, and nipple, in the absence of any known history of exposure to ionizing radiation or arsenic, and without any known alterations in immune surveillance or hereditary disorders that are known to increase one's risk of BCC. BCC of the nipple-areola complex is very rare. Only 24 cases were reported in the literature and the most of these cases arose in men. Most of the cases were treated with wide excision. We report on a case of BCC of the nipple-areola complex in a 73-year-old man, treated with total mastectomy with low axillary node dissection. (J Korean Surg Soc 2007;72:143-146)