Abstract
Craniopharyngiomas (CP) ectopic localizations off the midline outside the sellar-suprasellar region are very rare and most often associated to secondary tumor dissemination, being primary ectopic CPs extremely unusual. We herein report the first case of a primary ectopic craniopharyngioma within the orbit. A 15-year-old patient presented with progressive right ptosis, proptosis, and retro ocular pain. Magnetic resonance imaging (MRI) showed a predominantly cystic lesion, occupying the superolateral right corner of the orbit, outside the intraconal space with no evidence of intracranial involvement. An endoscopic transorbital eyelid approach was adopted. Pathology report disclosed a primary ectopic craniopharyngioma.