A 4-day-old baby was admitted with a large posterior fontanelle encephalocele. The baby was the third child of a consanguineous marriage. Two older siblings, 5 and 3 years old, were normal. The baby had a small head with a circumference of 30 cm only and an encephalocele with a circumference of 37 cm. The baby was active and there was no other neural tube defect or any other congenital anomalies. Noncontrast CT scan of the head with bone window showed a large posteriorly located encephalocele above the occipital bone, containing a small amount of brain tissue and a large volume of CSF. The baby was operated on in the lateral position and the encephalocele was excised. The dural defect was closed directly. The baby had an uneventful postoperative recovery. Twenty months later, the baby was well with no gross neurological deficits.
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