Amniotic fluid embolism (AFE) results from the passage of fœtal and amniotic fragments into the maternal circulation, occurring mostly within minutes before or after delivery. Although maternal and fœtal mortality of AFE remains high (about 40%), AFE should no longer be considered as having an ineluctable fatal course. Diagnosis is often made upon clinical presentation but histological confirmation is difficult owing favorable outcome and because an autopsy has not been performed. Identification of squamous cells in the maternal circulation could not confirm the diagnosis because of their possible maternal origin. High plasma level of insulin-like growth factor-binding protein-1 (IGFBP-1) has recently been identified as a biomarker of amniotic fluid passage into the maternal circulation and might therefore be used to confirm the diagnosis when lung tissue histology is not available. Treatment of AFE remains supportive with a special focus on correction of the coagulopathy and search for acute core pulmonale. In this later case, physicians should consider initiating an extracorporeal life support when facing a patient with refractory shock. Finally, caution is needed with the use of recombinant factor VIIa in this context.