Beckwith-Wiedemann syndrome is characterized by macroglossia, abdominal wall defect and neonatal hypoglycemia. This was first described by Beckwith in 1963 and by Wiedemann in 1964. Its acronym, ""EMG syndrome"", is derived from Exomphalos, Macroglossia, and Gigantism, the syndrome's major triad of clinical findings. About 200 cases have been reported in the literature. The feature seen most commonly now is macroglossia and it has found in up to 82% of patients with this syndrom. Orofacial maldevelopment associated with untreated macroglossia includes increased facial length with severe malocclusion, an open bite, and a widening of dental arches. So in the presence of profound macroglossia in the Beckwith-Wiedemann syndrome, acompelling need exists for surgical reduction of the tongue as early in the life as possible. Recently we experienced a case of huge macroglossia, treating with marginal glossectomy with the aid of carbon dioxide laser. So we report herein our case with a brief review of literatures.