Beckwith-Wiedemann Syndrome (BWS) is a rare genetic disorder that causes developmental defects as well as an elevated risk of malignancies. Macroglossia, or an enlarged tongue, is a common symptom of BWS that may have a negative influence on a person's quality of life. The aim of this systematic review is to look at the present state of knowledge about the repercussions of macroglossia, as well as the influence of the timing of surgical resection, or glossectomy, in the treatment of severe cases of macroglossia (Ref. 35). Keywords: macroglossia, Beckwith-Wiedemann syndrome, glossectomy.
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