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Objective: Iniencephaly is a rare neural tube defectwith consisting of a defect in the occipital bone,spina bifida of many vertebrae, and retroflexion of the head on the spine. In majorty of cases it is a le-\thal condition., Methods: We present the first case of iniencephalywith large bronchogenic cyst diagnosed prenatally., Results: At 19 week's gestation showed that fetalcardiac activity was present with normal placentaand amniotic fluid, fetus had occipital bone defect, anencephaly, retroflexion of the head, abnormally short cervicothoracic spine and posterior mediastinal unilocular anechoic cyst. Therapeutic abortion was induced., Conclusion: Iniencephaly is a rare condition during prenatal life. When diagnosed early in pregnancy amultidisciplinary approach is firmly suggested.