Although tumors of the central nervous system in children constitute the second most prevalent tumor type of childhood, spinal cord tumors account for less than 10% of pediatric central nervous system tumors. The most common are intramedullary, although they can be found in the extradural compartment or as intradural extramedullary masses. Extradural tumors can arise from bony elements, the meninges, or soft tissues. Neuroblastomas and sarcomas are frequently encountered along with bone tumors. Intradural extramedullary tumors can be meningeal or from distant sites and include meningiomas and schwannomas; most tend to be benign. Intradural intramedullary tumors, neuronal or glial, can be derived from neuroepithelial tissues. For the intramedullary tumors, astrocytomas represent around 60% of tumors, ependymomas 30%, and developmental tumors 4%.Such tumors require a multidisciplinary approach to ensure optimal patient outcomes. Spinal cord tumors most often present with pain followed by motor regression, gait disturbance, sphincter dysfunction or sensory loss, torticollis, and kyphoscoliosis. Treatment is based on tumor type, but surgical resection is the mainstay. Predictors of outcome include the histological grading, extent of resection, and neurological status at the time of surgery.