A 37-year-old male was referred with a huge renal mass and multiple lung lesions. Abdominal and chest computed tomographic scan revealed a 11×15×17. 5 cm right renal tumor and multiple lung metastases. Neither tumor was enhanced, and no lymph adenopathy was detected. Percutaneous needle core biopsy was performed and the tumors were diagnosed as Wilms' tumor histologically supported by immunohistological positive staining to WT-1 and CD56. Since lung metastases were detected and the renal tumor reached the abdominal aorta on the left side, presurgical systemic chemotherapy (ifosphamide＋ carboplatin＋etoposide ; ICEx 4 courses) was performed. The renal tumor and the lung metastases were markedly decreased in size and subjected to a radical nephrectomy. Histological examination confirmed the diagnosis of Wilms' tumor without anaplastic change categorized as mesenchymal type. As an adjuvant therapy, irradiation to renal bed (40 Gy/20 fr) and bilateral lung (12 Gy/8 fr) were performed. Six months after the end of the therapy, no evidence of recurrence was detected.