Intramedullary spinal cord tumors (IMSCTs) are rare neoplasms of the central nervous system associated with severe neurological deterioration, morbidity, poor quality of life, and even death. The most common IMSCTs that we encounter are ependymomas, astrocytomas, and hemangioblastomas. Surgery remains the initial treatment of choice for IMSCTs. New and interesting therapies are currently under investigation and the extent to which they augment our current treatment paradigms remains to be seen but with the advent of novel therapies (nanomedicine, localizable therapies, and cell targeting) the future appears promising for lesions not amenable to gross total resection and a surgical cure. While complete resection is not always achievable, we must remain tempered by the paramount importance of our patient's neurological function, for it is their wellbeing and quality of life outcome that is the root purpose of all of the aforementioned endeavors.