The amniotic band syndrome is a collection of fetal malformations associated with fibrous bands that appear to entangle or entrap various fetal parts in utero, leading to deformation, malformation, or disruption. This syndrome is given many names yet follows a clearly defined clinical pattern. Misdiagnosis and inappropriate family counseling are chronic features. This article reviews the clinical features of the amniotic band syndrome, its epidemiology, and the status of prenatal and neonatal diagnosis. The spectrum of malformations associated with amniotic bands is summarized and illustrated. Major etiologic theories are examined. It is recommended that the clinician involved in the delivery of any infant manifesting elements of this unusual combination of defects seek specialized consultation in the pursuit of an accurate and precise diagnosis and appropriate genetic counseling.