Abstract
BACKGROUND: In neurosurgical practice, we rarely encounter hybrid nerve sheath tumors (HNST) in the orbit. We recently had a patient of this rare tumor. We believe that this is the first report where we resected the tumor transcranially. CASE DESCRIPTION: A 54-year-old male presented with the left proptosis and intraconal tumor of 43 mm. We performed fronto-orbital craniotomy to resect the tumor mass. His proptosis completely improved and discharged home with a modified Rankin Scale of 1. CONCLUSION: Transcranial resection of orbital HNST was a safe and effective way to treat. Since we do not have much data regarding this rare tumor, we need to accumulate more cases.