Hepatoblastoma is a rare, malignant liver tumour of childhood. Until the mid 1980s only around 30% of patients were cured, but with modern chemotherapy, and of course surgery, the cure rate is now at least 70%. This dramatic improvement in survival has been achieved by the national and international cooperation of paediatric oncology centres. The International Society of Paediatric Oncology Liver Tumour Group, in contrast to most other groups, has used preoperative chemotherapy in all patients, followed by delayed surgery. The group has also developed a novel staging system, called PRETEXT (PRE Treatment EXTent of disease), based on the anatomy of the liver and radiological findings at diagnosis, to try to predict resectability and outcome.