Primary extramammary Paget's disease of the axilla is a rare variant with the capability of mimicking other more common conditions. We present a case of a 65-year-old woman with inflammatory skin lesions of both axillae clinically unresponsive to long-term conventional topical therapy. We decided to excise and reconstruct the resulting soft tissue defect of the major lesion by using a thoraco-dorsal artery perforator-based Limberg's flap. The histopathological examination showed intracellular mucin, signet cells, and glandular structures, the typical pattern of the EMPD. This was a very interesting case of a per se rare condition.