The Chiari Malformation is a neural tube defect involving the posterior cranial fossa and the hind brain, characterized by herniation of the posterior fossa contents below the level of the foramen magnum. Four types were described by Hans Chiari in the late 19th century. Of these, Type III is exceedingly rare. We report the case of Chiari III malformation in a 24-week fetus by the integration of radiologic and autopsy findings which was conspicuous by the absence of high cervical spina bifida and the presence of occipital squama defect.