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Abstract Background Meningoencephalocele is a rare congenital anomaly that is characterized by herniation of brain tissue through a defect in skull. Generally, it could be divided by anatomical location of defect to occipital and frontoethmoidal. The exact etiology of this condition is unknown but many theories have been postulated. The condition is usually seen at birth but can be identified prenatally. Case presentation A newborn was brought to the hospital after a normal non-complicated vaginal delivery done by the ambulance personnel. The newborn had a bulging mass on the posterior aspect of the head. Therefore, he was admitted and neurosurgical consultation was done for further evaluation. Currently the patient is following up in well baby clinic as well as the neurosurgery clinic for normal development and milestones acquisition. Conclusions This case presents the opportunity for junior healthcare professionals to learn about a group of congenital neurological disorder in the content of a rare case presentation.