A 37-year-old man with the chief complaints of lumbago and fever is presented. Laboratory data showed the extreme elevation of alfa feto-protein (AFP), human chorionic gonadotropin (hCG) and lactate dehydorgenase (LDH). Computed tomography (CT) scan revealed a huge retroperitoneal tumor with multiple pulmonary nodules as well as left supraclavicular and left axillary lymph nodes enlargement Although he was suspected the testicular tumor with metastasis, he had no testicular abnormalities including tumor and microlithiasis. Therefore, he was diagnosed as a retroperitoneal extra-gonadal germ cell tumor, which had poor prognosis because of multiple metastasis and the tremendous increase of hCG. Although he was treated with three cycles of bleomycin, etoposide, and cisplatin (BEP), he achieved partial response and no normaHzation of tumor markers. After three cycles of BEP, he was treated with four cycles of paclitaxel and ifosfamide plus cisplatin (TIP) immediately. During chemotherapy, he was treated with his peripheral blood stem cell transplantation (PBSCT) as well. After the completion of two regimens' chemotherapies, all his tumor markers returned to be normal However, retroperitoneal tumor, left supraclavicular and axillary lymphnodes still remained. He underwent three operations including retroperitoneal lymphnode dissection with nephrectomy, left supraclavicular and axillary lymphnodes removal, respectively. All specimens had no viable cells, histologically. The patient has been quite well and free of disease for 24 months. It is concluded that even if far-advanced germ cell tumor is discovered, a more promising prognosis could be expected with intensive and aggressive treatment such as our case. © 2010 Japanese Urological Association.