Meningoencephaloceles of the skull base most commonly occur as a sequela of head trauma or they can more rarely be congenital malformations. Several types of encephalocele exist depending on anatomic features and localisation. Clinical presentation and symptoms can vary. Different methods of management and repair of the concurring skull base defects have been described and ranging vary from endoscopic to open surgical approaches. We report the case of a 56-year-old Caucasian woman with the diagnosis of right sided spontaneous transethmoidal meninoencephalocele. Clinical presentation of this rare pathology, methods of diagnostic and management and its outcome are presented. Spontaneous skull base meningoencephaloceles are rare entities, without clear underlying etiologies. Multidisciplinary management is recommended. The transnasal endoscopic approach provides a wide skull base exposure with more advantages and less morbidity in comparison with the conventional open approach.