Abstract: The genetic mechanism of the acute respiratory distress syndrome (ARDS) has been widely studied. Genetic variations influence the susceptibility and outcomes of ARDS via affecting the function of genes and are considered as the potential biomarkers and therapeutic targets. Nevertheless, the innate relationship between genetic variations and the pathogenesis of ARDS remains obscure. This review summarizes the latest studies on this topic concerning the influence of genetic variations on the inflammatory response, microvascular function, and coagulation mechanism, in hopes of bringing some illumination for the future studies.