A 15-year-old girl presented with left occulomotor nerve palsy and was found to have a space occupying lesion of the sellar region with invasion of the left cavernous sinus. A transsphenoidal approach lead to subtotal removal of a solid tumor with some remnants in the cavernous sinus and revealed the diagnosis of trabecular juvenile ossifying fibroma (JOF). A repeat magnetic resonance imaging was obtained within 1 month that showed intrasellar recurrence and growing tumor in the cavernous sinus. Therefore, a combined transsphenoidal and transcranial approach was performed to more aggressively remove the tumor. Subsequently, adjuvant proton radiotherapy was performed. JOF of the trabecular type is a rare fibro-osseous lesion of the craniofacial skeleton almost exclusively occurring in the maxilla or the mandible. To our knowledge, this is the first case of this tumor entity presenting as a sellar mass.