Cephaloceles represent herniated intracranial content through a skull defect.

Atretic cephaloceles are involuted cephaloceles containing meninges and neural crests and represent about 50% of all cephaloceles.

The majority of reported cases occurred in the parietal region, although some reports suggest equal incidence on both sides of the lambdoid suture (parietal and occipital).

An increased incidence of intracranial anomalies has been reported with atretic cephalocele, and these include Dandy-Walker syndrome, Walker-Warburg syndrome, and holoprosencephaly, among others.

Our patient went on to have surgical excision of the cephalocele. Histopathology found meningeal tissue within.