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Amniotic band syndrome is a collection of congenital abnormalities caused by early amnion rupture. It occurs in approximately 1:1200 to 1:15000 live births. The infant we are reporting was as result of dizygotic twins pregnancy of 34-years old woman in first delivery than born by cesarean section. In clinical examination there were multiple defects such as: Anencephaly, anophthalmia, cleft lip-palate, nose malformation and limb anomalies. Involved infant died an hour after birth, because of severity of anomalies. This case was reported because of rarity and severity signs of this syndrome and its occurrence in one twin from dizygotic twins.