Historically, spinal cord tumors represent approximately 15% of all primary central nervous system (CNS) tumors, whereas the newest classification of these intramedullary spinal cord tumors (IMSCTs) encompasses only 2–4% of all CNS tumors. The more prevalent intramedullary lesions include astrocytoma and ependymoma, which are thoroughly described elsewhere in this book. Astrocytoma followed by ependymoma are the most common IMSCTs found in children, while in adults ependymomas are more frequent. The less common IMSCTs are hemangioblastoma and spinal cord metastases, which are much more common in the adult population. Exceedingly rare lesions, such as lymphoma, primitive neuroectodermal tumor (PNET), lipoma, and neuroenteric cysts, paraganglioma, ganglioneuroma, and oligodendroglioma—among others—have been documented in both the adult and pediatric literature and will be elaborated on in this chapter. Spinal cord hemangioblastoma, spinal cord metastases, and spinal cord cavernous angioma are thoroughly described elsewhere in this book.