Amniotic fluid embolism is a rare obstetric emergency in which amniotic fluid, fetal cells, hair, or other debris enters the maternal circulation. Amniotic fluid embolism is an incompletely understood obstetric complication unique to pregnancy presenting with the acute onset of hypoxia, hypotension and severe coagulopathy. Up to 86% of the cases are fatal, maternal death occurs within the first hour of symptom onset in 50% of the patients. It cannot be predicted nor prevented. The epidemiology of amniotic fluid embolism, frequency, pathophysiology, clinical presentation, histologic findings, diagnosis, differential diagnosis, possible treatment, mortality rate, neonatal survival are discussed in this review article.