Lingual tonsil hypertrophy is a common cause of persistent airway obstruction in patients with Down syndrome (DS) following adenotonsillectomy (T&A); however, little is known about the effect of lingual tonsillectomy (LT) on polysomnographic outcomes in these patients. Our objective was to describe changes in sleep-related respiratory outcomes following LT in children with DS and persistent obstructive sleep apnea (OSA) following T&A. Retrospective case series. We included all children with DS who underwent polysomnography before and after LT at a tertiary care center from 2003 to 2013. Nonparametric analysis of variables was performed. Forty patients with DS underwent LT; 21 met inclusion criteria. The mean age at surgery was 9.3 ± 4.3 years and 47.6% were female. The median apnea-hypopnea index (AHI) was 9.1 events/hour (range, 3.8 to 43.8 events/hour) before surgery and 3.7 events/hour (range, 0.5 to 24.4 events/hour) after surgery. The median improvement in overall AHI and the obstructive AHI (oAHI) were 5.1 events/hour (range, -2.9 to 41) and 5.3 events/hour (range, -2.9 to 41), respectively (P <.0001). The mean oxygen saturation nadir improved from 84% to 89% (P =.004). The mean time with CO2 > 50 mm Hg, central index, and percentage of rapid eye movement sleep were not significantly different. After surgery, the oAHI was <5 events/hour in 61.9% and ≤1 in 19% of patients. In children with DS, persistent OSA after T&A and lingual tonsil hypertrophy, LT significantly improved AHI, oAHI, and O2 saturation nadir. We recommend that children with DS should be evaluated for lingual tonsil hypertrophy if found to have persistent OSA following T&A. 4 Laryngoscope, 2016 127:520-524, 2017.