Extramammary Paget disease of the vulva is a rare condition that accounts for only 1-2% of vulvar malignancies and represents a frequent cause of misdiagnosis. It is most commonly seen in postmenopausal women. Clinically it is similar to Paget disease of the breast, appearing as red, well-demarcated eczematoid lesions, with slightly raised edges. A high degree of clinical suspicion is very important when evaluating these lesions in order to avoid misdiagnosis and delay of effective treatment. We present a case of vulvar Paget disease treated with vulvectomy.