UROD and Wilms tumor: A genotype/epigenotype-phenotype correlation has recently been established for BWS [17]: hemihypertrophy is strongly associated with upd(11)pat, exomphalos with ICR2 hypomethylation and CDKN1C mutations, and, most importantly, the risk of Wilms tumour is significantly higher in ICR1 hypermethylation and upd(11)pat than in the other molecular subgroups.