Extramammary Paget's disease (EMPD) is a rare cutaneous malignancy which mostly involves women and the most frequent site is labia majora. The most involved site in male patients is penoscrotal area. The diagnosis of EMPD depends on clinical presentation and pathohistological feature of the primary lesion. Surgical excision remains the treatment of choice. Metastasis implies poor prognosis and F-18 FDG PET/CT may help in survey for metastasis.