Summary

Beckwith-Wiedemann syndrome (BWS) is a congenital growth disorder characterised by abdominal wall defects, macroglossia and somatic gigantism. A number of associated features, including gastrointestinal and urinary tract polyps, have been described, but there are no previous reports of oral polyps occurring in this syndrome. We describe the first case of BWS presenting with an oral polyp. Clinicians should be alert to the possibility of BWS if other features of the syndrome are present, in children with oral polyps.