Wilms' Tumour accounts for 6% of all paediatric cancers. With modern multimodal therapy outcomes for patients is generally good (with an overall survival of >90%) and a reduction in treatment related morbidity. This review highlights recent developments risk stratification using histological grade and tumour stage. A rational approach to workup of these children is presented focusing on imaging interpretation. Management is discussed with emphasis on surgical technique and an update of chemotherapeutic strategies.