Objective: To determine the epidemiological, clinical, paraclinical characteristics and evaluate the result in treatment of hepatoblastoma (HB) at Department of Hematology-Oncology, Children’s Hospital 2 from January 2011 to end of May 2019. Method: Cases series study in combination of retrospective and prospective. Results: From January 2011 to end of May 2019, there were 67 patients diagnosed with HB. The mean age at diagnosis was 22.7 ± 22.9 months with the most prevalent age group ranging from 6 months to 3 years. The male to female ratio was 1.8/1. The most common clinical symptoms were abdominal distention and hepatomegaly, approximately 7.5% HB cases were detected accidentally by ultrasound. Nearly 60% and 80% patients presented with anemia and thrombocytosis at admission, respectively. Almost tumors were unifocal locating in the right lobe of the liver and 58.2% cases belonged to standard risk group. Assessing treatment protocol in 54 HB patients demonstrate that number of patients undergoing hepatectomy after preoperative chemotherapy accounted for 82%. The most frequent acuteside effects after chemotherapy were myelotoxicity and infection. The proportion of fatal and relapsed cases was 31.5% and 12.9% respectively. Overall and event-free survival rate of the study after 3 years was 65.7% and 68.1% individually. Conclusion: Hepatoblastoma is a rare malignant childhood disease, pretty good response to chemotherapy followed by surgery. However mortality rate is rather high compared to worldwide reasearchs. Thus development of liver transplant is necessary to improve survival rate.