Extramammary Paget's disease (EPD) represents an uncommon carcinoma that occurs mainly in the postmenopausal Caucasian women. Apocrine gland-bearing areas are predominantly affected.
The condition tends to occur as a slowly growing, invasive or noninvasive, mostly pruritic, lesion. In 25% EPD is associated with underlying internal malignancies mostly genitourinary, gastrointestinal or breast carcinoma.
Vulvar Paget's disease accounts for less than 1% of all vulvar malignancies. In our case the diagnossis of this rare carcinoma was delayed for 6 years.