Sepsis is a common disease the field of critical care medicine. It is easy to be complicated by acute lung injury (ALI) or even acute respiratory distress syndrome (ARDS), which lead to high mortality. Although domestic and foreign scholars have carried out a large number of research on the pathogenesis of sepsis-related ALI/ARDS, it has not been fully elucidated so far. Recent studies have found that mitochondrial DNA (mtDNA) may play an important role in sepsis-related ALI/ARDS. This article reviews the current role of mtDNA in the pathogenesis of sepsis-related ALI/ARDS, with a view to providing new clues and evidence for the treatment of sepsis-related ALI/ARDS.