Objective To investigate the diagnosis and treatment of hepatoblastoma in children. Methods The diagnosis, treatment and long-term follow-up of 12 children with hepatoblastoma during January 2007 and February 2010 were analysed. Results According to International Society of Pediatric Oncology (SIOP) Pretest staging system, there was 1 case of stage Ⅱ, 1 case of stage Ⅲ and 10 cases of stage Ⅳ.  All the 12 cases were treated with both chemotherapy and surgery. Both the case of stage Ⅱ and case of stage Ⅲ survived. Among the 10 cases of stage Ⅳ, 8 survived, 1 died of brain metastasis after drug withdrawl, and the other died due to no response to treatment. Conclusion Radical resection of tumor plays a key role in the treatment of hepatoblastoma in children. Chemotherapy is of great significance in the implementation of radical operation and prevention of tumor recurrence.