Giant encephaloceles are rare entities with only one case series and few case reports reported in the medical literature. Encephaloceles which reach a size larger than the head size are called giant encephaloceles. We report a case of a 6-month-old child who had giant encephalocele with delayed motor milestones in the form of inability to hold neck. Because of their enormous size, they pose a surgical challenge. Anesthetic implications include difficulty in securing airway without undue pressure on the sac. The baby underwent ventriculoperitoneal shunt followed by excision of the encephalocele sac with repair. We report this case to highlight the difficulties in the management of giant occipital encephaloceles.
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