Agenesis of spleen, Agenesis of vermis, Corpus callosum agenesis, Dandy walker syndrome

Introduction: Dandy Walker Syndrome (DWS) is a congenital brain malformation characterized by hypoplasia or absence of cerebellar vermis, cystic dilatation of fourth ventricle and hydrocephalus. It is frequently associated with other congenital anomalies. Associated central nervous system anomalies such as agenesis of corpus callosum and vermis are associated with poor prognosis. Association of DWS with congenital absence of spleen is life threatening condition and has been reported very rarely. Autopsy findings of DWS with association of congenital absence of corpus callosum, vermis and spleen are reported in a stillborn fetus of 28 weeks gestation and review of relevant literature was done.