Patients with Apert syndrome have mid-face hypoplasia associated with mandibular hypoplasia or other airway anomalies, which puts them at risk of obstructive sleep apnea (OSA). OSA is highly prevalent in patients with Apert syndrome and peaks in the first months of life, with a severity negatively correlated with age. Diagnosis of OSA cannot be made on symptoms or sleep questionnaires, which lack sensitivity and specificity. A polysomnography or respiratory polygraphy is thus necessary for OSA diagnosis and severity. Treatment strategy should be personalized and made by a pediatric expert multidisciplinary team and associates upper airway surgery, maxillofacial surgery, orthodontic treatment, and/or neurosurgery. Upper airway surgery constitutes the first-line treatment with various procedures depending on the patient’s age and the findings of a drug-induced endoscopy. This enables the identification of all obstructive sites. Upper airway surgery may consist of congenital piriform aperture dilatation, inferior turbinectomy/turbinoplasty, septoplasty, resection of the posterior part of the vomer to widen the nasopharynx, adenoidectomy, partial or total tonsillectomy, pharyngeal expansion, tongue base reduction, and/or supraglottoplasty. In case of persistent severe OSA without any possibility of an orthodontic or surgical treatment, continuous positive airway pressure (CPAP) may be proposed. This non-invasive treatment of upper airway obstruction is very effective and has the advantage that it may correct OSA while waiting for curative surgery. CPAP can be performed at home at any age. A long-term follow-up is mandatory in every patient with Apert syndrome because OSA may relapse during childhood. Indeed, OSA should be diagnosed and treated as early as possible to prevent the deleterious neurocognitive, behavioral, cardiovascular, and metabolic consequences of OSA. And finally, patients with Apert syndrome should be managed by an expert multidisciplinary team.