Wilms tumor is the most frequent pediatric renal cancer, it accounts for 6% of all pediatric cancers. Recent advances of multidisciplinary approach and tremendous efforts lead to an excellent outcome of the disease. With ongoing worldwide randomized controlled studies, tumor stage and histopathologic subtype have long been described as important prognostic factors. It has been suggested more recently the age, tumor size, genetic abnormalities, (especially loss of genetic material on chromosome 1p and 16q), and response to therapy may be incorporated in the risk stratification for protocol based therapies.

Urologist plays an important role as surgery stays at the main corner of both staging and management. With such a high cure rate already achieved, the main therapeutic goal in patients with non metastatic disease and with a tumor of favorable histology is to reduce the morbidity and late effects of the disease and treatment, without impairing the results. From this perspective, partial nephrectomy and laparoscopic surgery are interesting alternatives. Future protocols may provide nephron sparing and endourologic surgery as new prospects for treatment, therefore urologists will have to keep their hands working.