Here, we describe the case of a neonate suffering from respiratory distress complicated by pulmonary hemorrhage shortly after birth. The neonate experienced repeated dyspnea and acute severe hypercapnia (160-100 mmHg) that began on the 7th day after birth during invasive mechanical ventilation. Rescue high frequency oscillatory ventilation played a key role in maintaining gas exchange during these episodes. Subsequent bronchoscopy revealed tracheobronchial edematous mucosa with a cobblestone pattern, which we believe is the first report of this condition in neonates. Protrusion of the diffuse edematous mucosa led to airway stenosis and respiratory distress. Lesions of the tracheal mucosa should therefore be considered as one potential cause of neonatal respiratory distress.