Abstract
Giant encephalocele is a rare pediatric surgical entity that poses unique challenges. Few cases have been reported in the medical literature, and its cause is unknown. Factors that increase the likelihood of developing this pathology are radiation, infections, hyperinsulinemia, vitamin deficiencies related to neural tube closure defects, maternal smoking, alcohol, and anticonvulsants. A three-month-old female infant, with no factors associated with the current condition, with poor prenatal control, diagnosed with giant frontoethmoidal encephalocele, and with the presence of a frontal tumor of approximately 15 cm in its major axis, underwent surgery by a neurosurgeon and a plastic surgeon to correct the craniofacial deformity. The first approach was performed by removing the tumor with the closure of the meninges and skull. Then in the second surgical time, craniofacial reconstruction was performed by the plastic surgery team, using the same skin without the need for flaps of any other structure and without the presence of complications. The patient had no post-surgical complications. After seven years of medical follow-up, she has a normal staturoponderal and psychomotor development, no intellectual deficit, and adequate aesthetic results.