Cephalocele refers to defects in the skull and dura with extensions from intracranial to extracranial structures. Cephalocele is divided into four types which are meningoencephalocele, meningocele, atretic encephalocele, and gliocele. Encephalocele is a head's contents herniation through a defect in the skull. Meningocele is a herniation sac that contains cerebrospinal fluid and nerve elements. Meningoencephalocele is a prominent herniation of the meningeal part, nerve elements, and brain tissue in a sac that protrudes through a defect in the skull. In Southeast Asia, the incidence of meningoencephalocele is estimated to occur in 1 in 5000 live births. The occipital bone is the most common location of cephalocele. The neurological outcome of malformations that occur depends on the size of the sac formed, the nerve tissue involved, hydrocephalus, related infections, and other pathological conditions involved. Perioperative preparation must be well made by an anesthesiologist based on airway management, fluid balance, and hypothermia prevention. The main challenge of anesthesia in the management of the occipital meningoencephalocele is securing the airway. Pediatric patients have low functional reserve volume, and failure of tracheal intubation can cause hypoxemia, bradycardia, and even heart attacks. Improper positioning and limited neck extension can complicate endotracheal intubation.