BackgroundOccipital encephalocele is a rare congenital malformation, it is an anomaly of the brain and skull. It is defined as a defect of closure of the neural tube and it is associated with a hernia of the cerebral tissue outside the skull during the period of fetal development. Encephalocele affects between 0.4 and 4 cases per 10,000 births.The caseWe documented the case of a female Newborn from a full-term pregnancy and eutocic delivery, the baby was born eutrophic. She was admitted on the third day of life, followed for occipital encephalocele, and hospitalized in the neonatology department for specialized clinical and paraclinical monitoring while awaiting surgery. Surgery was performed in several stages, an occipital plasty with repositioning of the bulging area in the skull, removing protrusions, correcting deformities, and relieving the pressure that could interfere with the physiological development of the brain by a ventriculoperitoneal diversion of cerebrospinal fluid on the sixth day of life. This innervation involved the participation of different teams for optimal management. The patient did not develop any early postoperative complications and remained under observation in the neonatology department. His clinical evolution continued to improve in the days after the cure of the encephalocele.ConclusionThis case report demonstrates that folic acid intake during pregnancy can prevent the occurrence of the anomaly and that early repair significantly increases the child's vital and functional prognosis of the nervous system.