Beckwith – Wiedemann syndrome is congenital, genetic and epigenetic pathologies with low prevalence and diverse clinical
presentations. It is characterized by triad of omphalocele, macroglossia and gigantism. This syndrome has been widely studied with
a current emphasis on improvement of prenatal diagnostic techniques and a multidisciplinary approach towards treatment. We
report a case of BWS from Saudi Arabia, with unique presentations and misleading history which delayed diagnosis, due to cultural
and religion constraints.