Spinal cord ependymomas are the most common intramedullary spinal cord tumor in adults. At present, the best outcomes in treating these tumors are seen with complete surgical excision, while preserving neurologic function. Surgical dissection of these tumors from within the spinal cord can be tedious and complex, and requires experience and a dedicated team of treating physicians and surgeons. Advances in the care of this disease will come from improved early differential diagnosis, with imaging and possibly liquid biopsy. Improving the safety of surgical resection by enhancing preservation of neurologic function will also be needed.