Amniotic fluid embolism (AFE), also referred to as anaphylactoid syndrome of pregnancy, is a rare obstetric emergency that may manifest itself at any time during pregnancy. AFE is believed to occur when the constituents of amniotic fluid enter the maternal circulation, leading to varying degrees of multiorgan compromise. AFE was first described in 1926, gaining widespread recognition in 1941. This article describes the pathogenesis of AFE, including theories of its immunological mediation available in the literature. The most current diagnostic and treatment modalities are discussed, including several novel therapies. A case report of a 40-year-old parturient who suffered probable AFE following amniotomy, with the development of cardiopulmonary compromise, neurologic involvement, fetal distress, and coagulopathy, is outlined. The patient survived emergency cesarean delivery and hysterectomy with no residual physiologic deficits.