Fetal iniencephaly is an uncommon neural tube anomaly characterized by fixed retroflexion of the head, an occipital defect, and cervicothoracic rachischisis. Polyhydramnios and elevated levels of alpha-fetoprotein are present in most cases. Fetal iniencephaly can be detected on antenatal ultrasonography. If undiagnosed prenatally, it may result in a difficult delivery as well a multitude of postnatal problems for both the patient and the family. Early antenatal diagnosis is therefore important. We present a case of iniencephaly detected on ultrasonography and fetal magnetic resonance imaging.