Wilms tumor is the most common tumor of renal origin found in children. In the last 50 years, remarkable progress has been made in the treatment and understanding of children with Wilms tumor. Through the development of multiagent chemotherapy and cooperative pediatric interdisciplinary groups conducting large randomized controlled clinical trials, survival has improved dramatically. In the next century it is expected that 80% of children with Wilms tumor will be long-term survivors. Therapy is progressing towards a risk-based management based not only on stage and histology but also incorporated genetic markers. This article reviews progress to date and possible future directions in the treatment of Wilms Tumor.