Perlman syndrome is an autosomal recessive syndrome due to mutation in the gene encoding DIS3L2 exoribonuclease (RNAase); DIS3L2 has been shown to lead to IGF2 overexpression, as seen in the Beckwith–Wiedemann syndrome and is strongly associated with tissue overgrowth and Wilms tumor development (84). Here, IGF2 is linked to Nephroblastoma.