Several inherited syndromes have predisposed children to hepatoblastoma, including Familial Adenomatous Polyposis (FAP) with inactivating mutations of the APC gene, Beckwith-Wiedemann syndrome (BWS) with defective imprinting of the IGF2-H19 locus on chromosome 11p15, and Trisomy 18 (Edwards Syndrome) 2. This evidence concerns the gene IGF2 and Beckwith-Wiedemann syndrome.