Introduction: Neurally Adjusted Ventilatory Assist (NAVA) is an emerging mode of mechanical ventilation that is aimed at optimizing patient-ventilator synchrony. The properties and initiation of each breath adjust based on the patient's current physiologic state and ventilatory demand. This dynamic approach to ventilation allows the patient to completely control each breath while also reducing fatigue and lung injury. NAVA accomplishes this by using a modified nasogastric tube with electrodes situated at the gastric end to measure electromyelogram (EMG) potentials generated by the diaphragm. The magnitude of each potential is used to formulate each breath delivered by the ventilator. There is no single strategy in managing mechanical ventilation in patients with a Congenital Diaphragmatic Hernia (CDH), but aims should be at protecting against lung injury. NAVA has only previously been described in post operative care of CDH. In this report, we demonstrate how NAVA mechanical ventilation was applied to a patient with a CDH both pre and post-operatively. Case Description: Our case index is a healthy 14 month old female who presented to the emergency department with acute respiratory distress. The patient required immediate intubation with mechanical ventilation. Chest x-ray demonstrated an Anderson Catheter tip projecting over the left upper quadrant of the abdomen suggestive of gastrothorax. During the first 24 hours, the patient had discomfort and asynchrony with synchronized intermittent mechanical ventilation (SIMV) and was requiring increased amounts of sedative medications. After having asynchrony with SIMV pressure control pressure support (PS), SIMV pressure regulated volume controlled PS, and SIMV volume controlled PS, the patient was switched to NAVA mechanical ventilation. The patient appeared more comfortable and sedative medication was weaned. NAVA was continued until the patient was transferred to the operating suite for surgical closure of the left anterior Bochdalek diaphragmatic hernia via a gortex patch. Once the patient recovered from general anesthesia, she was placed back on NAVA ventilation. On post-operative day one, the patient was extubated and successfully weaned to room air. Discussion: The diaphragm is the principle inspiratory muscle in humans. It is innervated by phrenic motor neurons found in the ventral horn of levels C3-C5 of the cervical spinal cord. The respiratory function and control of the diaphragm is dependent on these phrenic motor units. The number of units recruited to generate a given force is dependent on the contractility and fatigue of the diaphragmatic muscle fibers. This is the specific EMG activity that is detected by the NAVA catheter and transmitted as an electrical activity of diaphragm (Edi) signal to the ventilator. For instance, if a patient's central nervous system sends a signal to recruit more motor neurons, a greater EMG potential will be measured by the NAVA catheter. The catheter transmits an Edi signal to the ventilator which results in increased pressure delivered to the patient. NAVA mechanical ventilation requires intact respiratory drive and detection of EMG signal from the diaphragm. In this case, the patient had intact respiratory drive, but also had a known diaphragmatic defect. A trial of NAVA was chosen in efforts to optimize mechanical ventilation, reduce amount of sedation, and also reduce possible lung injury. After placement of the EMG recording catheter, a chest x-ray confirmed that the gastric end of the catheter was within the portion of the stomach herniating through the diaphragm. Conclusion: In this patient with diaphragm pathology, NAVA allowed the patient to self control for optimal ventilation. Despite the defect in this case of CDH, the diaphragmatic EMG potentials were transmitted successfully resulting in delivery of appropriately synchronized ventilation.