Skull base chordomas represent very interesting neoplasms, due to their rarity, biological behavior, and resistance to treatment. Their management is very challenging. Recently, the use of a natural corridor, through the nose and the sphenoid sinus, improved morbidity and mortality allowing also for excellent removal rates. Prospective analysis of 54 patients harboring a skull base chordoma that were managed by extended endonasal endoscopic approach (EEA). Among the 54 patients treated (during a 72 months period), 21 were women and 33 men, undergoing 58 procedures. Twenty-two cases (40%) were recurrent and 32 (60%) newly diagnosed chordomas. Among the 32 newly diagnosed chordomas, a gross total resection was achieved in 28 cases (88%), a near total (>95% of tumor) in 2 cases (6%), a partial (>50% of tumor) in 2 cases (6%). Among the 22 recurrent chordomas, resection was complete in 7 cases (30%), near total in 7 (30%), and partial in 8 (40%). The global gross total resection rate was 65% (35/54 cases). Four patients (11%) recurred and 4 (11%) progressed within a mean follow-up of 34 months (range 12-84 months). Four patients (11%) were re-operated; one patient (1.8%) died due to disease progression, one patient (1.8%) died 2 weeks after surgery due to a massive bleeding from an ICA pseudo aneurysm. CSF leakage occurred in four patients (8%), and meningitis in eight cases (14%). No new permanent neurological deficit occurred. The EEA management of skull base chordomas requires a long and gradual learning curve that once acquired offers the possibility of either similar or better resection rates as compared to traditional approaches while morbidity is improved.