Obstructive sleep apnea (OSA) in the pediatric sickle cell disease (SCD) population can promote nightly hemoglobin oxygen desaturation, which increases the risk of central nervous system insult and may impair cognitive function. Adenotonsillectomy can ameliorate OSA symptoms, but its effect in children with SCD has not been fully investigated. We reviewed the effects of adenotonsillectomy in thirteen children with SCD by comparison of pre and post-adenotonsillectomy polysomnography (PSG) parameters. Significant reduction in hemoglobin oxygen desaturation, decreased apnea-hypopnea index, and increased rapid eye movement sleep occurred after adenotonsillectomy. Adenotonsillectomy promotes improvement in sleep quality in children with SCD and PSG-confirmed OSA.