RESUMEN
Introduction: and importance: An encephalocele is a type of congenital neural tube defect defined by herniation of intracranial contents via a cranial defect. When an encephalocele is greater than the size of the head, it is referred to as a "giant encephalocele." The occurrence of encephalocele has been documented to be 1-4 instances per 10,000 live births. Surgery is challenging. Case presentation: A 1-month-old baby boy sustained a huge swelling on the back of his head since birth, and it increased gradually over time. On examination, he had a huge occipital swelling measuring about 20 × 15 × 17 cm in size. A diagnosis of giant occipital encephalocele was established. Surgical excision and repair was done. After 1 month, he developed obstructive hydrocephalus and a ventriculo-periotoneal shunting was performed. On regular follow-up, he is in a good state of health. Discussion: Surgery imposes challenges for the anaesthesiologists and neurosurgeons due to its complex site, enormous size, intraoperative blood loss, and prolonged anaesthesia. A team approach is necessary for its successful treatment. Conclusions: Based on our experience, we would like to deliver following recommendations in the surgical management of giant encephalocele. Surgery should be done quickly to ensure good prognosis. Proper positioning, efficient intubation, infections and sepsis control should be emphasized. Also, fortification of food with folic acid, as well as increased education and awareness of women on the need for antenatal care may also decrease the risk of this disease.