A 64-year-old man was admitted with dyspnea and edema of the left lower extremity to the Department of Medicine, and referred to our department presenting with anuria and scrotal edema. Physical examinations revealed a large nodular tumor extending from his penis to the lower abdomen. He was diagnosed with penile tumor that had invaded into the retroperitoneal space. Histological findings revealed extramammary Paget's disease. Radiation therapy, low dose chemotherapy, and hormone therapy were performed, but he died of invasion and multiple metastasis of the tumor 5 months later.