We conducted an evidence-based review of information bout amniotic fluid embolism (AFE). The estimated incidence of AFE is 1:15,200 and 1:53,800 deliveries in North America and Europe, respectively. The case fatality rate and perinatal mortality associated with AFE are 13–30% and 9–44%, respectively. Risk factors associated with an increased risk of AFE include advanced maternal age, placental abnormalities, operative deliveries, eclampsia, polyhydramnios, cervical lacerations, and uterine rupture. The hemodynamic response in AFE is biphasic, with initial pulmonary hypertension and right ventricular failure, followed by left ventricular failure. Promising therapies include selective pulmonary vasodilators and recombinant activated facto Vlla. Important topics for future research are presented.