A 35 year female with progressive proptosis of right eye since childhood with diminution of vision presented with a swelling extending into the upper eyelid with a pulsatile proptosis. Globe deviated inferionasally with a visual acuity of finger counting at 1 meter. 3D-NCCT/MRI- Complete aplasia of the right sphenoid wing with an intraorbital temporal lobe meningoencephalocele. Dura and orbital tissue were inseparable due to adhesions. Redundant dura was plicated and sutured to the surrounding dura & falxcerebri (> 270° periorbital circumference) with no additional mesh construct. Post op improvement in vision to 6/9 with minimal enophthalmos was seen.

Keywords: Sphenoid Wing Dysplasia; Orbital Encephalocele; Neurofibromatosis-1; Encephalocele; Proptosis.