Morning Glory Syndrome is a very rare condition. It is characterized by an enlarged and funnel-shaped optic disc excavated and is associated with other craniofacial anomalies such as hypertelorism, cleft lip, cleft palate, and basal encephalocele. CaseReport. A case of a 30 year-old man who came for neurosurgical evaluation after a magnetic resonance imaging (MRI) scan that showed spheno-ethmoidal encephalocele is reported. Conclusions. There is a high association between basal encephalocele,midface abnormalities and Morning Glory Syndrome. A neurosurgical, ophtalmological and endocrinological evaluation must always be done. The patients present with ophtalmic signs and/or strabismus or poor vision. An image study should always be performed to delineate the extent of the lesion and a complete hormone screening should be carried out to exclude pituitary deficiency.

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