Amniotic fluid embolism (AFE) is a rare and often fatal obstetric complication, characterized by sudden cardiovascular collapse, dyspnea, seizures, mental alteration or coma and laboratory and clinically dia-gnosed disseminated intravascular coagulation (DIC). Patients reaction is typically biphasic with initial pulmonary hypertension and right ventricular failure, followed by left ventricular failure during or immediately right after labor. Early recognition of AFE is critical to a successful survival. Aggressive shock management is needed in collaboration with an anesthesiologist. Several aspects of the condition remain a controversy. This review critically examines, from the best available evidence, the current knowledge regarding the epidemiology, pathophysiology, dia-gnosis, and available treatment of AFE. This dia-gnosis still determines perinatal morbidity and mortality and potential permanent neurological symptoms for surviving patients.