The authors give a brief introduction on the subject of mucocele and emphasize the rarity of the ethmoid-sphenoid location (55 cases listed by Lundgren in 1961). The principal clinical features are discussed which are: ocular and periocular and sometimes, occipital pain; ocular symptoms, often bilateral, of compression of the optic nerve and of the 3rd and 4th cranial nerves; and only rarely, slight signs of nasal obstruction or of hypopituitarism. Of the various surgical approaches, the lower trans nasal septum operation seems to be the best choice. A case report of a patient with copious spontaneous rhinorrhea followed by meningitis is reported. The authors note that this symptom, not previously encountered in the literature, permitted a diagnosis to be made before the lesion became bilateral, which prior to this case, had always been observed. The lower trans-septum surgical approach with the aid of a microscope was employed, permitting total removal of the mucocele and tamponage of the cerebrospinal fluid fistula.