ABSTRACTA report of case of a patient with Beckwith‐Wiedemann syndrome is presented. The macroglossia associated with the syndrome necessitated long‐term dental follow‐up to prevent and treat problems with occlusion, speech, and esthetic appearance that might develop. Early intervention might be required if feeding or respiratory difficulties, resulting from the macroglossia, arise. The diagnosis of the syndrome should alert the dentist to the increased incidence of intra‐abdominal malignancies and the need for periodic obse