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Intramedullary spinal tumors are uncommon intra-axial lesions, which can be either primary or metastatic. Primary tumors arise from cell of spinal cord and account for 2–4% of all intrinsic tumors of the central nervous system, being much less common of brain tumors. They are slow-growing tumors, so symptoms precede diagnosis by an average of 2 years. Metastatic lesions usually originate from lung and breast tumors and are usually diagnosed within 1 month from symptom onset. Pain and weakness are the most common presenting symptoms. Magnetic resonance imaging represents the gold standard technique to study the spinal cord tumors, and first-line treatment is surgical resection, but it is not always curative. In selected situations, watchful waiting can be considered. Chemotherapy and radiation are considered, but controversy exists. Novel treatment options must be developed to supplement partial resection and recurrence.