OBJECTIVE: To analyze the clinical characteristics, immunohistochemistry, and treatment outcomes for skull base chordomas and the correlation between extent of resection/pathology type and outcomes. METHODS: The clinical materials of 77 consecutive patients with skull base chordomas were analyzed retrospectively. Follow-up data were available in 66 patients, ranging from 6 to 142 months (mean, 59.6 months). Outcome in survival was assessed by the overall survival (OS) and progression-free survival (PFS). Outcome in function was evaluated by Karnofsky performance score. RESULTS: Total or near-total tumor resection was achieved in 25 cases (32.5%), subtotal in 32 cases (47.6%), and partial resection in 9 cases (11.7%). Gamma knife radiosurgery was used in 22 patients (33.3%). Forty-two of the 77 patients had immunohistochemistry results and the rates of positive staining for cytokeratin, epithelial membrane antigen, vimentin, S-100 were 100%, 92.9%, 83.3%, and 88.1%, respectively. The PFS rates at 3, 5, and 8 years were 82.6%, 45.0%, and 18.2%, respectively. The OS rates at 3, 5, and 8 years were 89.2%, 70.9%, and 45.5 %, respectively. Less tumor resection and dedifferentiated pathology were risk factors for worse OS and PFS (P < 0.05). Among the 43 currently surviving patients, the mean Karnofsky performance score before the surgery and at the last follow-up were 87.3 and 82.4, respectively. CONCLUSIONS: Aggressive surgical resection should be performed for skull base chordomas, considering certain postoperative functional status. Immuno-histiochemical study is helpful in differential diagnosis. The combination of aggressive surgical resection and gamma knife radiosurgery for skull base chordomas may obtain favorable outcomes.