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The literature discussing the long-term outcome after resection of upper cervical spinal cord tumors is limited. The purpose of this study was to review the progression-free survival (PFS), overall survival (OS), and long-term outcomes in a consecutive series of 51 patients with upper cervical spinal cord tumors who underwent surgery at our institution between 2005 and 2010. Patient outcome were evaluated using the Japanese Orthopaedic Association score (JOA) and the McCormick functional schema. Follow-up data was collected completely and the median follow-up time was 6.1 years. Gross total resection (GTR) was performed in 27 patients (52.94%) and subtotal resection (STR) in 24 patients (47.06%). Progression-free survival and overall survival at 5 years was 88.23% and 92.16%, respectively. Good prognosis was defined as 74.51% based on JOA scoring. The univariate analysis showed that patients over 60y, tumors located higher than C2, tumor size greater than 4 cm as well as malignant tumors and subtotal resection were factors indicating a poor prognosis. However, the multivariate regression analyses showed only the level of tumor and tumor size were independent risk factors for a poor prognosis. The gold standard treatment for intraspinal tumors is gross total resection and follow-up should be focused on patients with a high risk of poor prognosis.