Only a few larger maternally inherited deletions in patients with BWS including H19 in addition to the ICR1 have been described recently by Baskin et al. [19] and one paternally inherited deletion in a mosaic state, which interestingly lead to atypical Silver-Russel syndrome (SRS, an intrauterine growth retardation syndrome) [22,23]. Here, H19 is linked to Silver-Russell syndrome.