Amniotic band sequence (ABS) results from in utero entrapment of fetal parts by fibrous bands, which leads to malformations that can affect multiple organ systems including the limbs, craniofacial regions and trunk, and central nervous system. These bands can adhere to anatomical structures of the fetus and cause vascular disruption which results in the amputation of the involved anatomic part. We present two cases of ABS both of which presented with craniofacial defects associated with ABS; one with apical encephalocel and extremity defects, the other one with schizencephaly with extremity defects and cleft lip & palate. ABS may represent itself not only with extremity defects but atypical forms in cranial vault. ABS must be kept in mind and sonography must be detailed when rare deformities like schizencephaly and extremity defects identified.