We describe a case of histologically confirmed malignant melanoma of the nipple. The rare occurrence of these lesions accounts for the relative lack of criteria for standard surgical treatment. After a conventional workup including mammography, chest X ray, bone scan, liver ultrasonography and cytologic smear of the lesion, we used specific radiolabeled monoclonal antibody and external photoscanning to differentiate melanoma from Paget's disease. The patient underwent wide local excision of the lesion and axillary node dissection, and tumor control is optimal since she has no evidence of disease after 5 years of follow-up.
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