Extramammary Paget’s disease is a rare malignant tumor that frequently presents in areas rich in apocrine glands, most often affecting the skin of the perianal area: the labia minora, clitoris, scrotum, penis, skin of the lower abdomen, and inguinal folds. Tumors localized outside the perianal region are relatively rare. We present a clinical case of a 70-year-old patient with a tumor localized on the skin of the right axillary region, with clinical, histological, and immunohistochemical characteristics. The diagnosis of extramammary Paget’s disease with atypical localization is relatively difficult to reach in time, as tumors are often mistakenly considered as inflammatory skin lesions, and the appropriate treatment may therefore be delayed.