Amniotic fluid embolism is one of the most catastrophic complications of pregnancy. First described in 1941, the condition is exceedingly rare and the exact pathophysiology is still unknown. The etiology was thought to be embolic in nature, but more recent evidence suggests an immunologic basis. Common presenting symptoms include dyspnea, nonreassuring fetal status, hypotension, seizures, and disseminated intravascular coagulation. Early recognition of amniotic fluid embolism is critical to a successful outcome. However, despite intensive resuscitation, outcomes are frequently poor for both infant and mother. Recently, aggressive and successful management of amniotic fluid embolism with recombinant factor VIIa and a ventricular assist device, inhaled nitric oxide, cardiopulmonary bypass and intraaortic balloon pump with extracorporeal membrane oxygenation have been reported and should be considered in select cases.