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Background: Encephalocele is defined as a herniation of the neural tube due to skull defect. anterior encephaloceles are common in males while the occipital form occurs in females. We present in this article a case of a giant occipital encephalocele and provide a literature review on the subject.

Case description: We report a case of 8 months old female, the third child of a family of 3 children with notion of consanguinity of third degree, the mother had no notion of taking medication during pregnancy, this one was followed to term. the delivery was by caesarean section. on the clinical examination the infant mobilizes the 4 limbs, no sensitive or motor deficits, no occulomotor disorders, cranial perimeter at 37 cm, the general examination does not find any other malformations. we operated the patient with ablation of the encephalocele, the postoperative continuations were simple, the evolution was marked by the stability of the cranial perimeter, and the infant was hospitalized for 5 days, then declared out with a good postoperative evolution.

Conclusion: Occipital encephalocele is considered as a very common form of neural tube defect the diagnosis is based on neuroimaging techniques; we believe that its Management includes full investigations for optimum surgical plan.

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