Apocrine adenocarcinoma of the skin is a rare and, most of the time, clinically misdiagnosed malignant adnexal tumour. A 66-year-old female patient presented with an asymptomatic swelling of the left nipple that on pathological evaluation was confirmed as an apocrine adenocarcinoma. Surgery is to be considered as the mean treatment for such a lesion and the diagnosis is always difficult to establish. To our best knowledge, this is only the second reported apocrine adenocarcinoma arising from a nipple and the first presenting with Paget's phenomenon.
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