Dandy-Walker malformation is a rare congenital defect characterized by agenesis or hypoplasia of the cerebellar vermis with cystic dilatation of the fourth ventricle, and enlargement of the posterior fossa. We present a rare case of Dandy-Walker malformation associated with a meningocoele and lissencephaly, in a female baby born alive but died soon after. There was agenesis of the cerebellar vermis and lateral displacement of cerebellar hemispheres exposing the dilated fourth ventricle, which communicated with a posterior cranial fossa cyst. Cerebral hemispheres with incompletely formed sulci and gyri were observed. A midline foramen in the occipital bone was seen through which the meningeal layer herniated from posterior cranial fossa. Complete antenatal examination, karyotyping & ultrasonography may help in early diagnosis and decision making.