Acute cor pulmonale (ACP) is one of the serious complications of acute respiratory distress syndrome (ARDS). Its occurrence can not only cause congestion of the systemic circulation but also affect the contraction and diastolic function of the left heart, further aggravating circulatory failure. At present, significant progress has been made in the lung protective ventilation strategy for ARDS, but the mortality of patients with moderate to severe ARDS remains high. The author reviews ACP in ARDS patients in the respects of epidemiology, pathophysiology, diagnostic methods, and mechanical ventilation strategies.