Acute respiratory distress syndrome (ARDS) is a clinically defined entity describing the severity of diffuse alveolar injury caused by direct or indirect injury to the lung. Pathophysiology, clinical course and outcome of ARDS depend on the underlying cause, the severity of the disease and co-morbidities. Pulmonary function tests show restrictive lung disease, which is characterised by a reduction in lung compliance and functional residual capacity, resulting in marked ventilation-perfusion inequality. Current ventilator strategies aim to minimise ventilator-induced lung injury by targeting mechanical ventilation between the lower and upper inflection point of the pressure volume curve. This includes recruitment manoeuvres and the use of high PEEP to open the atelectatic lung and the use of permissive hypercapnia and the limitation of peak inspiratory pressure below 35 cm H2O to avoid overinflation. The clinical benefit of newer modes of ventilatory support such as inverse ratio ventilation, high frequency oscillatory ventilation, surfactant replacement, prone positioning and inhaled nitric oxide has yet to be determined in children.