Key Points: 1. Children with Beckwith-Wiedemann syndrome (BWS) are at increased risk of obstructive sleep apnea (OSA) 2. While management of OSA in children with BWS traditionally involves noninvasive measures, surgical intervention may be required 3. Surgical intervention for OSA in BWS children includes tongue reduction, tongue-lip adhesion and/or lingual frenectomy, tonsillectomy, adenoidectomy, uvulectomy, lingual tonsillectomy, tongue base reduction, and epiglottopexy 4. In the present study, surgical intervention for OSA in children with BWS significantly improved mean apnea-hypopnea index and nocturnal oxygen saturation nadir on postoperative polysomnography 5. Surgical intervention may improve or resolve OSA in children with BWS