Acute respiratory distress syndrome (ARDS) is a common clinical disorder caused by a variety of direct and indirect injuries to the lung, characterized by alveolar epithelial and endothelial injury resulting in damage to the pulmonary alveolar-capillary barrier. The cardinal clinical feature of ARDS, refractory arterial hypoxemia, is the result of protein-rich alveolar edema with impaired surfactant function, due to vascular leakage and vascular dysfunction with consequently impaired matching of ventilation to perfusion. Since its first description in 1967, considerable knowledge concerning the pathogenesis of ARDS has been obtained, however, a plethora of questions remain. Better understanding of the pathophysiology of ARDS has lead to the development of novel therapies, pharmacological strategies, and advances in mechanical ventilation. However, lung-protective ventilation is the only confirmed option in ARDS management improving survival, and few other therapies have translated into improved oxygenation or reduced ventilation time. But despite improvement in our understanding of the therapy and supportive care for patients with ARDS, mortality remains high. It is the purpose of this article to provide an overview of the definition, clinical features, and pathogenesis of ARDS, and to present and discuss therapeutic options currently available in order to effectively treat this severe disorder.