Intramedullary spinal cord tumors are rare central nervous system tumors with unique challenges due to the eloquence of the surrounding tissue. Their treatment and prognosis is largely dependent on tumor histology and patient functionality. The introduction and advancement of microsurgical techniques have made surgery the mainstay of treatment for intramedullary tumors. Tumors that are well demarcated (e.g., ependymomas, hemangioblastomas) can be resected for cure, while more infiltrative tumors (e.g., high-grade astrocytomas) are typically managed with biopsies or limited resections in order to minimize the significant risk of damage to the spinal cord. The use of more aggressive surgical resection for astrocytoma is controversial but may have an increasing role in select cases. The use of intraoperative neurophysiologic monitoring and intraoperative ultrasound may help guide the extent of surgery while minimizing damage to normal tissue. Advances in MRI technology have greatly aided the diagnosis and preoperative planning of intramedullary tumors. Further advances in intraoperative MRI may make this a useful tool in guiding extent of resection. Preoperative functional status is the most important predictor of neurologic outcome, while histology and extent of resection are the most important predictors of progression-free survival. The use of adjuvant radiation and chemotherapy is dependent on patient age and histology but is largely reserved for high-grade tumor histologies or systemic involvement. Children are particularly at risk of radiation-induced injury, and these cases may benefit from more focused stereotactic radiation where necessary. Further studies are needed to support new surgical strategies minimizing destabilization and to investigate new forms of adjuvant therapy to minimize toxicity.