Amniotic fluid embolism is infrequent but potentially lethal obstetrical complication. Altough the understanding of this entity has improved over the years, amniotic fluid embolism is still associated with high maternal and perinatal morbidity and mortality. The exact cause as well as the patophysiological mechanisms have not yet been fully clarified. It is believed that the mechanism of the disease is associated with the immune response of the mother to fetal antigenes. While it was previously considered that the autopsy finding of the fetal components in the mother`s circulation is pathognomonic, that is now abandoned as a diagnostic method. The diagnosis is clinical, based on the exclusion of other possible causes. Currently, there is no specific diagnostic serological or histological test. Treatment is supportive, focused on maintaining vital parameters and is performed in intensive care units. The outcome of the treatment is better in case of early start of life support measures. There are no specific risk factors that would justify any changes in current obstetric practices in order to avoid amniotic fluid embolism or reduce the risk factors.