A Dandy-Walker malformation is an anatomical triad characterized by a large posterior fossa, agenesis of the cerebellar vermis, and dilation of the 4th ventricle. Up to 90% of patients will have hydrocephalus secondary to obstruction of fourth ventricular outflow through the foramen of Luschka and Magendie. The differential diagnosis of Dandy-Walker malformation includes Blake’s pouch cyst, a cystic structure originating within the roof of the 4th ventricle that herniates into the cisterna magna, and posterior fossa arachnoid cysts, which typically displace both the cerebellum and 4th ventricle anteriorly and demonstrate a propensity to enlarge. Brain MRI is the best diagnostic imaging modality to evaluate posterior fossa anatomy and cystic structures. Not all posterior fossa cysts require treatment. Indications for surgical intervention include enlargement, symptomatic mass effect and/or hydrocephalus.