Obstructive sleep apnea (OSA) is a sleeping breathing disorder. In children, adenotonsillar hypertrophy remains the main anatomical risk factor of OSA. The aim of this study was to assess the current scientific data and to systematically summarize the evidence for the efficiency of adenotonsillectomy (AT) and orthodontic treatment (i.e., rapid maxillary expansion (RME) and mandibular advancement (MA)) in the treatment of pediatric OSA. A literature search was conducted in several databases, including PubMed, Embase, Medline, Cochrane and LILACS up to 5th April 2020. The initial search yielded 509 articles, with 10 articles being identified as eligible after screening. AT and orthodontic treatment were more effective together than separately to cure OSA in pediatric patients. There was a greater decrease in apnea hypoapnea index (AHI) and respiratory disturbance index (RDI), and a major increase in the lowest oxygen saturation and the oxygen desaturation index (ODI) after undergoing both treatments. Nevertheless, the reappearance of OSA could occur several years after reporting adequate treatment. In order to avoid recurrence, myofunctional therapy (MT) could be recommended as a follow-up. However, further studies with good clinical evidence are required to confirm this finding.