Acute pancreatitis (AP) is one of the common diseases of the digestive system mainly characterized by acute inflammation of the pancreas and acinar cell destruction, and it can often develop into severe acute pancreatitis (SAP). Acute respiratory distress syndrome (ARDS) is one of the most common and serious complications of SAP and a common cause of death in patients with SAP. In recent years, there have gained new understanding of the pathogenesis, diagnosis, and treatment of SAP-related ARDS, however, a variety of drugs that have been shown to prevent ARDS in SAP animal models have not improved the prognosis of SAP-related ARDS patients in clinical researches. At present, the treatment of SAP-related ARDS still follows the general treatment principles of ARDS, such as treatment of primary disease, lung protective ventilation strategy, prone position ventilation, early short-term use of neuromuscular blockers and extracorporeal membrane oxygenation (ECMO), and so on. However, the influence of high intra-abdominal pressure, intestinal function failure, abdominal incision, and drainage tube on the treatment measures should also be considered for patients with SAP-related ARDS. This article reviews the recent progress in diagnosis and treatment of SAP-related ARDS.