Jarcho-Levin syndrome is a rare disorder characterised by defects in vertebral and costal segmentation of varying severity. Respiratory complications are the main cause of death or severe comorbidity due to a restrictive rib cage. A 3 months old infant with Spondylocostal dysostosis and associated bronchomalacia experiencing severe asynchrony during the weaning process is reported. The Neurally Adjusted Ventilatory Assist (NAVA) mode was used to improve adaptation to mechanical ventilation after Vertical Expandable Prosthetic Titanium Ribs (VEPTRs) were implanted. The synchrony achieved with the NAVA mode allowed a decrease of the sedoanalgesia he received. A follow-up CT scan showed a reduction in the volume of the posterobasal atelectasis. The evolution of this patient suggests that the combined use of VEPTR for thoracic expansion and ventilation using NAVA can favour the global improvement. This mode could be an option to consider in selected patients with difficult weaning from mechanical ventilation in paediatric intensive care units.