Amniotic band syndrome (ABS) is a rare condition associated with vascular constrictions from fibrous bands entanglement of fetal body parts. We report a case of a neonate with amniotic band syndrome diagnosed at birth. The neonate presented with hypoplasia, vestigial, and gangrenous distal third of the right leg. Below the knee- amputation was done 48 hours after delivery, with a satisfactory outcome. This report captures a literature review on the pathogenesis, clinical presentations and treatment of ABS.