Wilms' tumor (nephroblastoma) is a subject which continues to challenge clinicians in their attempts to achieve the best survival of their patients, while minimizing morbidity. Overall survival of over 85% of all children can now be achieved using combination therapy with chemotherapy, surgery, and in some cases radiotherapy. Recent reviews of two multi-institutional, multinational trials (the International Society of Pediatric Oncology and the National Wilms' Tumor Study Group) are discussed in this article, in which their current approaches to therapy are presented. In addition to these clinical advances, the genetic and molecular features of Wilms' tumor continue to provide insight into the biology of nephroblastoma and into neoplasia in general. A recent review of these aspects of the field is provided.