Chordomas are rare, slow growing tumors of the axial skeleton, which derive from the remnants of the fetal notochord. They can be encountered anywhere along the axial skeleton, most commonly in the sacral area, skull base and less commonly in the spine. Chordomas have a benign histopathology but exhibit malignant clinical behaivor with invasive, destructive ant metastatic potential. These tumors present with headaches and diplopia.

Skull base chordomas are very challenging to treat. Clinically there are two subsets of chordoma patients with distinct behaviors: some with a benign course and another group with an aggressive and rapidly progressive disease. There is no standard treatment for chordomas. Surgical resection and high dose radiation treatment are the mainstays of current treatment. The endoscopics techniques have improved the resection grades and the morbimortalitiy, against the transfacials and transcranials approaches (Figure 1). Pre and postoperative MRI (Figure 2). Intraoperative image.