Only 2% of childhood tumours occur in the spine and spinal cord; yet these tumours account for a disproportionate degree of morbidity in children with cancer. Spine and spinal cord tumours frequently initially present with non-specific symptoms such as back pain and clumsiness and are therefore often associated with a prolonged period between symptom onset and diagnosis. Many children present repeatedly to healthcare services before a diagnosis is made. and appropriate imaging is often only instigated once a child has developed neurological deficits. Unfortunately, despite treatment, these deficits are often only at best partially reversible. This article reviews the pathology and presentation of spine and spinal cord tumours in children and advises on the appropriate assessment of a child who may have a spine or spinal cord tumour. The principles underlying the management of these tumours are discussed and the management strategies for individual tumour types summarised.