Cyclophosphamide is considered to be a bladder carcinogen and there are many reports of secondary bladder cancer, while only a few cases of upper urothelial cancer have been described. A 59-year-old man, who had received cyclophosphamide therapy for malignant lymphoma, was suffering from gross hematuria and consulted our institute. Computerized tomography (CT), intravenous pyelography (IVP) and retrograde pyelography (RP) revealed a left renal pelvic tumor. Urinary cytology showed class V and radical left nephroureterectomy was performed. Histopathological diagnosis of the left renal pelvic tumor was transitional cell carcinoma, invading the renal parenchyma. He is free from recurrence eight months after surgery. To our knowledge, this is the third case of cyclophosphamide-induced upper urothelial carcinoma reported in Japan, and the twelfth reported in the English literature.