Intramedullary spinal cord tumors (ISCT) are rare tumors requiring multidisciplinary care in an expert center. Here, we report a single-center experience including 247 patients with ISCT: 134 ependymomas, 54 astrocytomas and 59 hemangioblastomas. Preoperative evaluation and surgical considerations are discussed to share our principles in managing these patients. Ependymomas are largely benign lesions (95% of WHO grade II) for which total resection (91% rate of gross total resection (GTR)) without neurological impairment (79% of patients remained stable or improved) is the goal in order to ensure long-term oncological control (94.5% overall survival at 5 years, excluding anaplastic ependymomas). On the other hand, astrocytomas are more frequently high-grade tumors (30% of WHO grade III or IV) for which partial resection (60% of cases) is often necessary to preserve neurological status (48% of patients deteriorate at one year), leading to higher rates of recurrence (60% recurrence rate at 5 years for grade III astrocytomas). Lastly, spinal hemangioblastomas require a specific microsurgical resection with particular attention to the vasculature in order to ensure en bloc resection (95% GTR), allowing excellent neurological results (12% of patients improved, 86% remained stable). Altogether, these results demonstrate that ISCT can be managed surgically with good functional outcome, while oncological results will mostly depend on the histopathological grading.