Effective diagnosis, staging, and multimodality therapies dramatically decreased the morbidity and mortality of children with Wilms Tumor. Increasing awareness of the disease, biology, genetics, and epidemiology improved assessment of clinical syndromes and led to more risk-based treatment. The role of tumor imaging is being defined, and primary exploration with nephrectomy remains of value to confirm histological diagnosis and disease stage. Primary chemotherapy with delayed resection is evolving into the preferred approach for large inoperable tumors, bilateral disease, and those tumors with extensive intravascular involvement. Current therapeutic trials will be nonrandomized and emphasize biological tumor features for future patient stratification. A selected group of young patients with small localized favorable histology tumors will be treated with surgery alone.