Chordomas are uncommon malignant tumors of the axial skeleton, and the spheno-occipital location is the second most common to be involved after sacrococcygeal location. Vertebral body involvement is the least common. Chordomas originate from the bone and result in local bone destruction which is best evaluated by CT imaging.

MRI allows confident preoperative diagnosis. This case illustrates characteristic features of Chordoma which was confirmed on biopsy.