Spinal cord tumors are a challenge for patients and neurosurgeons because of the high risk of neurologic deficits from the disease process and surgical interventions. Spinal cord tumors are uncommon, and approximately 2%-3% of primary intra-axial tumors of the central nervous system occur in the spinal cord. Primary intra-axial tumors are usually derived from neuroepithelial tissue, especially glial cells. This often leads to a classic intramedullary mass differential diagnosis of ependymoma or astrocytoma, which together constitute up to 70% of spinal cord tumors. For example, ependymomas occur predominantly in adults, and astrocytomas (specifically pilocytic astrocytomas) occur predominantly in children. While that is an excellent starting point, in order to refine the differential diagnosis, the authors review the radiologic-pathologic features of specific neoplastic categories and entities recognized by the World Health Organization (WHO) in the 2016 WHO Classification of Tumours of the Central Nervous System and a few additional congenital-developmental entities. Radiologists can add value by providing a reasonable preoperative differential diagnosis for the patient and neurosurgeon, in many cases by favoring the most common conditions, and in other cases by identifying radiologic features that may point toward a less common entity. Some of the less common entities include intramedullary myxopapillary ependymoma, spinal subependymoma, and spinal hemangioblastoma. Whenever possible, the characteristic imaging features and locations of these tumors are explained or traced back to the underlying cell of origin and findings seen at histopathologic examination.See discussion on this article by Buch.