Chordomas are rare malignant tumors that develop from the residual of embryonic notochord. These tumors may be seen along the spine and have a local aggressive progression. Skull base chordomas often originate from the clivus as localization. These tumors are usually found to be overgrown when they are diagnosed. They are locally invasive and rarely develop distant metastasis. These chordomas cannot usually be completely removed due to their localization. Because these tumors are advanced at the time of diagnosis and are adjacent to important structures, they are among the tumors with high rates of mortality and morbidity. Surgery and/or radiotherapy is administered for the treatment.