Wilms tumour is uncommon but is one of the most common solid abdominal tumours in children. It can be both sporadic and associated with a number of genetic anomalies. Treatment and outcome are largely dependent upon stage and histological subtype. 5-year survival following multimodal therapy is >90% for the majority of cases who have low-stage disease and a favourable histological type. Combination therapy with vincristine and actinomycin D is the cornerstone of chemotherapy, with additional agents such as doxorubicin, cyclophosphamide and etoposide being added to drug regimens for more aggressive disease. Despite the good prognosis for the cancer, there are long-term risks associated with aggressive chemotherapy in childhood.