Amniotic constriction band , first described in 1832 by Montgomery1, is one term used to describe a wide range of associated congenital anomalies, including anular constrictions of multiple extremities, oligodactyly, acrosyndactyly, talipes equinovarus, cleft lip and cleft palate, and hemangiomas. Additional, less common clinical manifestations include complete absence of the limb, short umbilical cord, craniofacial disruptions, neural tube defects, cranial defects, scoliosis, and body-wall defects, such as gastroschisis and extrathoracic heart. Some of these manifestations are uncommonly noted at birth because they result in spontaneous abortion2-5. The prevalence of amniotic constriction band is approximately one in 1200 to one in 15,000 live births6,7. The prevalence rate for male infants has been reported to be 0.91 and, for female infants, 1.44. These defects are reported to occur 1.76 times more frequently among African-Americans as compared with Caucasians6. Evidence of familial involvement is extremely rare. Although temporal and geographic clustering has been reported, this phenomenon is not well understood8. The variability of presentation between patients, the unusual nature of this constellation of findings, and the lack of a consensus on etiology are all reflected in the fact that thirty-four different names have been used to describe this entity in the literature9. Most of the descriptive terminology used to describe this entity relates to the extremity manifestations; the central manifestations affecting the face and body have not typically been considered for nomenclature. The various names include amnion rupture sequence, aberrant tissue band syndrome, ADAM (amniotic deformity, adhesions, mutilations) complex2, constriction band syndrome, constriction ring syndrome, amnion disruption sequence, and Streeter dysplasia, among others.