Perioperative refractory ventricular fibrillation (RVF) refers to ventricular fibrillation that persists after standard cardiopulmonary resuscitation and multiple defibrillations, occurring within the high-risk window from the start of surgery to 24 hours postoperatively. The mortality rate is extremely high (85%-97%) and the rate of survival with intact neurological function is very low (only 5.6%). Currently, the specific epidemiological data for this condition is still lacking. As a manifestation of perioperative cardiac arrest, its incidence is (4.3-5.8) per 10, 000 cases, with significantly increased risk in emergency surgeries, infants, and elderly patients. The main risk factors include coronary artery abnormalities, concomitant heart failure or respiratory failure, and reperfusion injury related to cardiac surgery. The pathophysiological mechanism involves the synergistic effects of myocardial electrophysiological disorders, metabolic imbalance, and sympathetic storm. The key to prevention and treatment lies in implementing a stepwise intervention approach and initiating a rapid multidisciplinary collaboration process, including: promptly initiating high-quality mechanical CPR combined with dual sequential defibrillation (DSED), early identification of pulseless electrical activity (PEA) as a critical decision-making node for resuscitation; rational use of anti-arrhythmic drugs; early initiation of veno-arterial extracorporeal membrane oxygenation (VA-ECMO) support, and transfer to the hybrid operating room for coronary angiography/percutaneous coronary intervention (PCI), etc. This review systematically elaborates on the definition, epidemiological characteristics, risk factors, pathophysiological mechanism, current treatment principles, and research progress of perioperative RVF, aiming to provide a theoretical references for clinical practice.