Chordoma clivus is rare lesion that originates from remnants of the primitive notochord and represents one of the skull base tumors that is difficult to treat because of its location, invasiveness, and high recurrence rate, as well as the potential for extension around vital neurovascular structures. Although the surgical approach remains controversial, total tumor resection, combined with postoperative irradiation, is the mainstay of treatment and one of the most important factors for prognosis. We report a 40-year-old male patient who was visually impaired since the age of 9 years and had retinitis pigmentosa, with frame hemicranio left recurrent headache, nasal obstruction, and chronic rhinosinusitis without changes to the cranial nerves. A CT scan showed an expansive heterogeneous lesion, with its largest component in the sphenoid sinus and extending posteriorly, and showed bone destruction of the clivus, presenting with lateral extension and intimate contact with the cavernous sinus. Our study describes endoscopic nasal surgery for transsphenoidal excision of the lesion.