The majority of intramedullary spinal cord tumors in children are low-grade glial tumors. They become symptomatic with pain, neurologic deficits or spinal deformity. The diagnosis is most readily obtained using magnetic resonance imaging. The natural history is significant for slow progression of symptoms. Surgery is the best treatment and is also indicated to confirm the histological diagnosis. In case of a low-grade tumor or a vascular lesion such as hemangioblastoma or cavernoma, a total or near-total resection is attempted. For astrocytomas the resection almost always remains biologically incomplete, but a near-total resection is still associated with a long progression-free survival. Neurologic morbidity is relatively low during long-term follow-up but can be up to 30% for transient motor deficits. The risk for neurologic deterioration is higher for patients with pronounced dysfunction preoperatively. This is an important argument for early surgical resection. Surgery is performed using the spectrum of microsurgical techniques as well as advanced technology, e.g. lasers and intraoperative neurophysiological monitoring with motor evoked potentials. High-grade tumors are resected conservatively and treated with radiation and chemotherapy. The prognosis of high-grade glial tumors remains poor.