Chordoma is a rare, slow-growing, locally aggressive, primary bone tumor that arises from the skull base region in approximately 25-35% of cases. The therapeutic approach to chordoma has traditionally been surgery, followed by radiation therapy. The advent of charged particle radiotherapy has let us consider protons as the postoperative treatment of choice, but no controlled studies have yet confirmed the superiority of protons over photons. During January 2008, two independent researchers conducted a systematic review of the current data on the treatment of base of the skull chordoma C with proton therapy (PT) and, for comparison, with other irradiation techniques (conventional radiation therapy, ion therapy, fractionated stereotactic radiation therapy, and radiosurgery). Two hundred and ten reports in total were retrieved (81 concerning PT). According to the inclusion criteria, 47 articles were considered in the analysis. There were no prospective trials (randomized or nonrandomized) but just seven uncontrolled single-arm studies for PT, providing clinical outcomes for 416 patients in total; these reports were mainly related to advanced inoperable or incompletely resected tumors. The therapeutic approach to chordoma of the base of the skull has traditionally relied on surgical control. Radiation therapy has demonstrated to be a valuable modality for local control in the postoperative setting, particularly with the advent of charged particle radiotherapy. The use of protons has shown better results in comparison to the use of conventional photon irradiation, resulting in the best long-term (10 years) outcome for this tumor with relatively few significant complications considering the high doses delivered with this therapeutic modality.