Intramedullary spinal cord neoplasms are rare, accounting for about 4%10% of all central nervous system tumors. Despite their rarity, these lesions are important to the radiologist because magnetic resonance (MR) imaging is the preoperative study of choice to narrow the differential diagnosis and guide surgical resection. On contrast materialenhanced MR images, intramedullary spinal tumors almost always manifest as expansion of the spinal cord and show enhancement. Syringohydromyelia and cystic lesions are frequently associated with intramedullary tumors. Nontumoral cysts tend to be located at the poles of the tumors and do not enhance on contrast-enhanced MR images, whereas cysts within the substance of the tumor are considered tumoral cysts and typically demonstrate peripheral enhancement. Spinal cord ependymomas are the most common type in adults, and cord astrocytomas are most common in children. Both entities constitute up to 70% of all intramedullary neoplasms. A central location within the spinal cord, presence of a cleavage plane, and intense homogeneous enhancement are imaging features that favor an ependymoma. Intramedullary astrocytomas are usually eccentrically located within the cord, are ill defined, and have patchy enhancement after intravenous contrast material administration. Even with these characteristics, it may not be possible to differentiate these two entities on the basis of imaging features alone. Cord hemangioblastomas are the third most common type of intramedullary spinal tumor. Gangliogliomas commonly extend over more than eight vertebral segments. Paragangliomas and primitive neuroectodermal tumors have an affinity for the filum terminale and cauda equina. Other spinal cord tumors include metastatic disease, which is characterized by prominent cord edema for the size of the enhancing portion, and primary lymphoma.