Extramammary Paget's disease is an uncommon intraepithelial malignancy that usually presents as a well circumscribed, reddish eczematous patch on the apocrine-bearing regions such as the genital and perineal areas. It progresses slowly with local extension. However, it may become invasive and may be associated with internal malignancies. Extramammary Paget's disease has high local recurrence rates after complete surgical excision and therefore, long-term follow-up is necessary. Extramammary Paget's disease of the axilla is extremely rare, with few reported cases. We report here a case of extramammary Paget's disease of the axilla in a 68-yr-old man, with a review of the literature.