Introduction: Occipital encephalocele is a brain malformation that has been remotely associated with Dandy-Walker; only case reports and very small series have been published so far; therefore, their behavior and management are still under investigation. The goal of the present case-based review is to provide a summary of the state of the art in this association.
Methods And Results: The pertinent literature has been reviewed, and an exemplary case has been reported (an 11-month-old female with Dandy-Walker malformation and occipital encephalocele).