Hepatoblastoma (HB) is the most common liver malignancy in children. HB usually occurs in the first 3 years of life. The incidence is slowly but steadily increasing at a rate of 1.2-1.5 cases/million/year, partly due to prematurity and very low birth weight [1, 2]. The development of adjuvant, neo-adjuvant chemotherapy as well as the new surgical resection methods significantly improved the patients’ survival rate in the last decades . Despite these significant achievements in HB treatment, the prognosis of patients with metastasis and those who are at a stage of pretreatment extent of disease (PRETEXT) IV remains unfavorable . Thus, novel targeted therapy strategies against HB are highly needed. To achieve this goal, a better characterization of the molecular genetics and signaling pathways underlying HB pathogenesis is imperative.