Iniencephaly is the rarest form of neural closure defects. It is characterized by incomplete closure of vertebral arches and/or bodies; significant shortening of the spinal column, rotation and retroflexion of head. By the careful examination of the fetus by ultrasound, it could be detected at the end of the first trimester. However, contribution of the real time 3 dimensional (3-D) Ultrasonographic evaluation is unclear, it could be utilized for the differential diagnosis of other neural tube defects and Klippel-Feil Syndrome. In this manuscript we reported two cases of iniencephaly who were diagnosed at late first and early second trimester and contribution of real time 3-D scan.

Keywords: Iniencephaly, craniorachischisis, prenatal diagnosis, real time 3-D scan