Objectives/hypothesis: To examine outcomes following midline posterior glossectomy (MPG) plus lingual tonsillectomy (LT) for the treatment of significant obstructive sleep apnea (OSA) in children with Down syndrome (DS).Methods: Patients with DS who had persistent OSA following tonsillectomy and adenoidectomy (TA) and were relatively intolerant of positive airway pressure (PAP) therapy were evaluated by physical examination and sleep/CINE magnetic resonance imaging to determine the etiology of upper airway obstruction. Patients with relative macroglossia underwent MPG plus LT if required. Successful surgical outcome was defined as the resolution of OSA or the ability to tolerate PAP.Results: Thirteen children (8 male, 5 female), mean (standard deviation) age 14.2 (4.0) years underwent MPG plus LT. Fifty-four percent of patients were obese (Body mass index [BMI] > 95th centile) and 8% were overweight (BMI 85th-95th centile) preoperatively. All patients underwent pre- and postoperative polysomnography. Postoperatively, the obstructive apnea-hypopnea index fell significantly from 47.0/hour to 5.6/hour (P