Amniotic band syndrome (ABS) is not a commonly seen birth defect; however, it may have the potential to be severe and life-threatening requiring adequate attention. We present a severe case of amniotic band syndrome which encircled the head of the neonate tightly causing severe deformity. In this case report, the amniotic band encircled the head causing a severe bilateral Tessier 7 cleft. To our knowledge, this is the most severe type of ABS reported in the literature thus far.Level of evidence: Level V, risk / therapeutic study.