We describe a rare case of Dandy-Walker syndrome malformation and its ocular alterations. A female child, aged 1 year and 9 month, with central nervous system alterations (Dandy-Walker syndrome), associated with low visual acuity, megalocornea (13 mm diameter in right eye and 13.5 mm in the left), fundoscopy with 0.7 optic papilla excavation in both eyes (BE) and temporal paleness in BE, intraocular pressure of 16 mmHg in the right eye and 14 mmHg in the left and anteroposterior diameter of 20.55 mm in both eyes.