Presence of sphenoid wing dysplasia fulfills one of the diagnostic criterias of neurofibromatosis type 1 (NF1). This defect of the skull base can manifests as pulsating proptosis. Associated calvarial defects, especially away from the lambdoid suture are a much rarer presentation. This report describes the successful removal of plexiform neurofibroma followed by reconstruction of the dysplastic sphenoid wing in an NF1 patient. Later on, he underwent blepharoplasty for scarring of the upper eyelid. After successful management of the overall procedure in three stages, the patient achieved improved visual outcome along with acceptable cosmetic appearance.

Key words: Sphenoid wing dysplasia; neurofibromatosis; proptosis.