Neurally Adjusted Ventilatory Assist (NAVA) is an innovative mode of ventilation that delivers support in synchrony with the infant’s diaphragmatic electrical activity. This technology aims to improve patient–ventilator interaction, making its use in neonates with complex respiratory disease appealing. The use of NAVA in preterm infants is becoming more commonplace, but the specific role of NAVA in infants with established bronchopulmonary dysplasia (BPD), congenital diaphragmatic hernia (CDH), and those requiring extracorporeal membrane oxygenation (ECMO) support remain less clearly defined. Few studies have directly compared NAVA to conventional modes in those settings, but existing evidence suggests that NAVA may be feasible and beneficial, particularly by improving synchrony, reducing ventilator days, supporting post-extubation success, reducing sedation needs, and improving neurodevelopmental outcomes. We performed a narrative review of the current literature describing NAVA use in these three high-risk neonatal populations. Despite growing interest, high-quality evidence remains limited, and significant heterogeneity exists in the currently published literature. Further randomized prospective studies are needed to better define the role of NAVA in these complex patient populations and to evaluate long-term respiratory outcomes. This review aims to inform clinicians navigating the nuanced use of NAVA in advanced neonatal respiratory care.