A 70-year-old female was admitted to our hospital with a complaint of gross hematuria for two months. She had a 5 years history of eczematous vulvar skin eruption. With the diagnosis of T3N0M0 bladder cancer and anogenital Paget's disease, she underwent pelvic exenteration, vulvectomy and reconstruction of the vulva. Histopathological examinations and mucin stains revealed anogenital Paget's disease and invasive bladder cancer which extended to the clitoral skin and formed Pagetoid skin lesion. CEA and cyokeratin staining demonstrated identical expression pattern in bladder cancer cells and Paget's cells, which suggested both neoplastic cells originated from one progenitor cell.