Amniotic fluid embolism (AFE) is a life-threatening obstetric complication that arises in 2 to 8 of every 100 000 deliveries. With a mortality of 11% to 44%, it is among the leading direct causes of maternal death. This entity is an interdisciplinary challenge because of its presentation with sudden cardiac arrest without any immediately obvious cause, the lack of specific diagnostic tests, the difficulty of establishing the diagnosis and excluding competing diagnoses, and the complex treatment required, including cardiopulmonary resuscitation. Methods: We selectively reviewed pertinent literature published from 2000 to May 2013 that was retrieved by a PubMed search. Results: The identified risk factors for AFE are maternal age 35 and above (odds ratio [OR] 1.86), Cesarean section (OR 12.4), placenta previa (OR 10.5), and multiple pregnancy (OR 8.5). AFE is diagnosed on clinical grounds after the exclusion of other causes of acute cardiovascular decompensation during delivery, such as pulmonary thromboembolism or myocardial infarction. Its main clinical features are severe hypotension, arrhythmia, cardiac arrest, pulmonary and neurological manifestations, and profuse bleeding because of disseminated intravascular coagulation and/or hyperfibrinolysis. Its treatment requires immediate, optimal interdisciplinary cooperation. Low-level evidence favors treating women suffering from AFE by securing the airway, adequate oxygenation, circulatory support, and correction of hemostatic disturbances. The sudden, unexplained death of a pregnant woman necessitates a forensic autopsy. The histological or immunohistochemical demonstration of formed amniotic fluid components in the pulmonary bloodflow establishes the diagnosis of AFE. Conclusion: AFE has become more common in recent years, for unclear reasons. Rapid diagnosis and immediate interdisciplinary treatment are essential for a good outcome. Establishing evidence-based recommendations for intervention is an important goal for the near future.