Intramedullary spinal cord tumors are uncommon in children, and delayed diagnosis is common. Unlike the situation in adults, low-grade astrocytomas predominate followed by ependymomas and other gliomas. Recent technological advances, including MR imaging, intraoperative ultrasonography, the ultrasonic surgical aspirator, and the operative laser, have made radical resection of intramedullary tumors feasible. The long-term results of modern-day surgery using these techniques are only now becoming available, and the role of adjuvant radiotherapy or chemotherapy for the usual low-grade tumors remains unclear. The risk of spinal deformity after laminectomy in children and the effects of radiotherapy on the growing spine create special challenges in the treatment of pediatric intramedullary tumors.