Introduction: Skull base chordomas represent a challenge to the otolaryngologist because of its rarity, biological behavior, and resistance to treatment. Chordomas are known to originate from embryonic remnants of the notochord, presenting from the skull base to sacrum. Chordomas arising from skull base/clivus are typically locally aggressive with lytic bone destruction. The ideal treatment may be radiotherapy alone or combined with gross total resection, when feasible. Because of the risk of severe late complications, the radiotherapy dose is often limited and the probability of cure is low. Recently, the transnasal endoscopic approach has improved morbidity and mortality allowing also for excellent removal rates.