Extramammary Paget's disease is a rare cutaneous malignancy and frequently associated with an underlying adnexal carcinoma and perhaps with underlying internal ma1iycy. In our case, genita1 Paget's disease was followed by the discovery of renal cell carcinoma. We report a rare case of extramammary paget's disease of the penis and scrotum which associated with a renal cell carcinoma. (Ann Dermatol 11(1) 51~54,1999).