Wilms' tumour represents 8% of tumours appearing during childhood, and it is the more frequent malignant tumour of the urinary tract in children. A review of 7 tumours occurring in our Hospital Area over the last 11 years is made, evaluating their incidence, clinical presentation, diagnosis as well as therapeutical approaches and complications. We also conducted a review of the most recent related literature, paying special attention to the new lines of research in the field of the genetics. Likewise, we contribute the latest guidelines and suggestions by the NWTS with regard to diagnosis and treatment of Wilms' tumour.