Extramammary Paget's disease is a rare lesion, often involving the skin of the genital or perianal regions. Less commonly, it has been reported to affect the skin of the axilla. There are very few other cases of extramammary Paget's disease reported in the literature, and the appropriate use of newer techniques such as magnetic resonance imaging and sentinel lymph node biopsy in this setting is not well-studied. We present a case of extramammary Paget's disease of the axilla, and discuss the known literature regarding this rare disease.