Paget's disease of the breast nipple and areola complex represents a cutaneous manifestation of an underlying breast malignancy. The typical skin changes characteristic of breast Paget's disease are usually easily distinguished from those found of malignant melanoma. The rare finding of a pigmented cutaneous breast lesion mimicking melanoma presents a diagnostic dilemma. This case presentation describes a pigmented mammary lesion involving the areola that had undergone changes in diameter and pigmentation. The lesion was not associated with nipple drainage, nipple excoriation, dermal ulceration, or a palpable breast mass. The areola lesion was highly suggestive of a dermatologic malignancy such as malignant melanoma. As such, a biopsy was performed with initial findings suggestive of cutaneous melanoma. Upon more detailed analysis of a larger excision specimen, immunohistochemical staining was highly supportive of the diagnosis of breast Paget's disease. A total mastectomy with sentinel lymph node evaluation was performed along with immediate reconstruction. The final pathology revealed a small focus of ductal carcinoma in situ of the breast associated with the areola changes. Histologic and immunohistochemical analysis of a biopsy specimen were paramount in differentiating these two lesions.