OBJECTIVE: To review our experience with patients with macroglossia as a component of Beckwith-Weidemann Syndrome (BWS). DESIGN: Chart review of six patients treated with BWS. SETTING: Tertiary care teaching hospital. PATIENTS: Six patients diagnosed with BWS and macroglossia. INTERVENTIONS: Four patients underwent at least one surgical procedure to address their macroglossia. The surgical options and potential complications are discussed. RESULTS: Three patients who have undergone tongue reduction have a functioning tongue with normal mobility. Two patients have required tracheotomy as apart of their management and still have significant tongue enlargement. CONCLUSIONS: Macroglossia as a part of BWS may present a difficult management problem. Various methods of tongue reductions have been reported with mixed results.