The prognosis in nephroblastoma (Wilms' tumor) has been improved considerably by treatment protocols combining surgery, chemotherapy, radiation therapy, and, in some clinical trials, pre-operative chemotherapy. Cure is now achieved in most patients. All clinical trials have employed treatment strategies tailored to the individual risk of the patient, including the histological subtype of the tumor. In the National Wilms' Tumor Study (NWTS) of the United States these subtypes have been divided into two groups of tumors according to their "favorable" or "unfavorable" histology. At the Kiel Pediatric Tumor Registry we have devised a system which distinguishes three groups of tumors classified according to prognosis. The first group includes tumors with a favorable prognosis, even if only surgery is performed. These comprise congenital mesoblastic nephroma (CMN) and cystic, partially differentiated nephroblastoma (CPDN). The second group consists of tumors posing an intermediate risk, such as typical nephroblastoma and its histological variants characterized by variations in the relative proportions of the histological components. Fetal rhabdomyomatous nephroblastoma (FRN) is also included in this group. The third group comprises tumors of high risk such as anaplastic nephroblastoma, clear cell sarcoma of the kidney (CCSK), and malignant rhabdoid tumor of the kidney (MRTK). Since histological diagnosis plays a crucial role in the assignment of a patient to a particular type of treatment protocol, knowledge of the histological appearance of the various tumor types both with and without preoperative treatment is of utmost importance.