Dandy-Walker cyst associated with occipital meningocele is very rare. Only 12 patients have been reported. We report a female infant with Dandy-Walker cyst and occipital meningocele whose diagnosis was suspected antenatally by in utero ultrasonography. At birth, head circumference was normal for 37 weeks gestation. She underwent surgical repair of the occipital meningocele immediately after birth. The post-operative course was uneventful until the sixth day of life when progressive enlargement of the head with progressive ventricular dilatation was recognized. Communication between the posterior fossa cyst and the occipital meningocele was confirmed neuroradiologically; the occipital meningocele may have compensated for the increased intracranial pressure in fetal life.