Hydrocephalus, absence of the cerebellar vermis, and posterior fossa cyst continuous with the fourth ventricle are the main characteristics of this syndrome. About 4% of all cases of hydrocephalus are complicated by DWS. Associated disorders may include Aase-Smith syndrome 1, Aicardi, Coffin-Siris, cryptophthalmos, Ehlers-Danlos, Ellis-van Creveld, Jones syndrome, Joubert, Ruvalcaba-Myhre-Smith, Walker-Warburg, Meckel, Ritscher-Schinzel, and Veradi syndromes. Dandy-Walker syndrome associated with macrocephaly, facial anomalies, developmental delay, and brain stem dysgenesis inherited as an X-linked recessive trait was reported as a separate syndrome.