This syndrome is associated with increased susceptibility to tumors and growth abnormalities in children. A common cause of this syndrome is a mutation in an imprint control region <nowiki/>near the Igf2 gene. This imprint control region is normally bound by an insulator on the maternal allele, which represses an enhancer from acting on the Igf2 gene. This insulator is absent on the paternal allele and allows it access to the gene. Mutations in this imprint control region inhibit the insulator from binding, which derepresses enhancer activity on the maternal Igf2 gene. This abnormal derepression and increase in gene expression can result in Beckwith-Wiedemann syndrome.