Obstructive sleep apnea syndrome is one of the most frequent manifestations of upper airway obstruction. Several approaches have been proposed to solve this problem with the aim to avoid tracheotomy especially in children. We observed a child affected by Simpson-Golabi-Behmel syndrome presenting severe OSAS. He underwent tongue-base suspension using the Repose system with significant improvement of the polysomnographic parameters and of the clinical conditions. Tongue suspension with this technique effectively prevent tongue-base collapse, avoiding tracheotomy or increased morbidity caused by a more invasive procedure. Besides it does not interfere with the patient's cranio-facial growth and future occlusion.