BackgroundObstructive sleep apnea (OSA) has been associated with pulmonary hypertension (PH) through a complex and bidirectional relationship. The prevalence of OSA and other forms of sleep‐disordered breathing (SDB) among children with PH is not well understood. A better understanding could help elucidate contributing factors for PH, guide diagnostic testing, and inform therapies. We therefore determined the proportion of SDB codiagnosis and OSA risk factors among a cohort of children with PH.MethodsRetrospective chart review was performed for children 0−21 years old with PH who underwent polysomnography (PSG) between 1 January 2010 and 31 August 2020. The primary outcome was OSA occurrence and risk based on demographics, diagnoses, and PH classification. The secondary outcome was occurrence and risk of other SDB diagnoses based on PH classification.ResultsAmong 89 children identified, diagnoses included OSA (N = 79, 89%), central sleep apnea (N = 11, 12%), sleep‐related hypoventilation (N = 6, 7%), and non‐apneic hypoxemia (N = 28, 31%). Trisomy 21 diagnosis (N = 38, 43%) was associated with increased OSA risk (RR: 1.24, 95% CI: [1.09−1.42]) and age >12 months at PSG was associated with decreased OSA risk (N = 52, 58%, RR: 0.60, 95% CI: [0.45−0.81]). There was no difference in SDB risk based on Group 1 (N = 56, 63%) or Group 3 (N = 33, 37%) PH and no difference in OSA severity based on demographics and diagnoses.ConclusionOSA was diagnosed among a majority of children with PH, and other SDB diagnoses were identified in a significant proportion of children. These findings support routine screening for SDB with PSG in children with PH.