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Developmental anomalies of the skull base may present in childhood or as an adult. The most common pathologies include dermoid, encephalocele, and glioma. Encephaloceles may present as part of a syndrome. Other entities include infantile hemangiomas and teratoma. Endoscopic techniques provide a less invasive and morbid option for treatment. Proper evaluation and treatment is necessary to prevent complications such as meningitis.