Acute lung injury (ALI) and its severe form, acute respiratory distress syndrome (ARDS), is originated by multiple insults. ARDS is a significant source of morbidity and mortality in the critically ill patient population. The complex physiopathology of ALI/ARDS is characterized by inflammation, coagulation pathways dysregulation, injury of endothelial and epithelial barrier and pulmonary edema. Altogether provides a wide range of targets that offers multiple therapeutic options. In the last years, multiple preclinical and clinical studies have been performed for the treatment of ALI/ARDS; unfortunately the major part of these studies did not give any positive result. Nowadays, new therapeutically options and new administration ways have been tested, some of them with promising results. Herein, in this review, the results of several studies in animal models and clinical trials (phase I and II) are extensively revised, giving a summary of all the existing treatments with favorable options. Also, the research in ARDS has been focused in the last decade on the prevention of this disease, trying to decrease mortality and avoid the consequences of undergo this pathology. Recovery of lung alveolar epithelia, reabsorption of edema and regulation of inflammation and coagulation cascades are the best targets to try to resolve ARDS; new preclinical studies should be performed to develop novel therapies and clinical trials should be completed to confirm the obtained positive results.