We report on the use of single-agent docetaxel (100 mg/m(2) in children >10 kg, 3.3 mg/kg in children <10 kg), given as a 1-hour infusion at 21-day intervals in 5 children with relapsed or refractory hepatoblastoma. One patient achieved complete remission of pulmonary metastases after 2 courses of docetaxel and remains well 10 years later, after completion of 13 courses of docetaxel and whole-lung radiotherapy. One patient showed a partial response to docetaxel based on α-fetoprotein measurements. Docetaxel shows some activity in progressive hepatoblastoma in this small case series and is a potential drug for future study in this disease.