Conclusion: Dandy Walker variant may be asymptomatic and the images found may not indicate them as the cause of developmental disorders, due to its association with multiple syndromes and chromosomal abnormalities. Clinical presentation and prognosis depends on the related disorders, and a multidisciplinary approach is important, because the treatment depends on the symptoms presented. (C) 2016 Sociedad Chilena de Pediatria. Published by Elsevier Espana, S.L.U. This is an open access article under the CC BY-NC-ND license.