BWS patients carrying CDKN1C mutations show a higher frequency of abdominal wall defects and omphalocele (Brioude et al., 2015), whereas mice lacking of a maternal copy of the CDKN1C gene present a phenotype close to BWS with gastrointestinal tract abnormalities or exomphalos (Yan et al., 1997; Zhang et al., 1997). This evidence concerns the gene CDKN1C and omphalocele.