Giant Occipital Encephalocele with Chiari Malformation Type 3

Encephaloceles are rare embryological mesenchymal developmental anomalies resulting from inappropriate ossification in the skull through which herniate the intracranial contents of the sac. Occipital encephaloceles are described as giant when they are larger than the head from which they arise, and they pose a great surgical challenge. Herein, we present a case of a giant occipital encephalocele in a neonate with Chiari malformation Type 3 to highlight the problems encountered in its management and the outcome of the surgery.