A boy was born with Dandy-Walker syndrome associated with a giant occipital meningocele, cleft lip, and cleft palate. The meningocele was actually a component of the giant posterior fossa cyst which communicated with the fourth ventricle. A cyst-peritoneal shunt achieved a considerable decrease in the size of the meningocele, but decubital ulcers developed due to restricted head movement caused by the occipital lesion. Cranioplasty removed a wide area of the inferior occipital bone, and the boundary between the superior occipital and parietal bones was thinned to allow free bending of the bone flap. The meningocele was removed totally in the third operation, but infection of the wound and pneumonia developed, causing death. The coexistence of Dandy-Walker syndrome and occipital meningocele, together with midline facial anomalies, may suggest a later pathogenesis of Dandy-Walker syndrome than previously believed.