Holocord intramedullary low-grade astrocytomas in children and adolescents - involving most or all of the cervical and thoracic spinal cord - are a rare finding. Most of the tumors seem to be pilocytic astrocytomas. Surgical management strategies might not be as clear as in small and circumscribed intramedullary tumors. On the basis of 20 previously published cases and 3 own patients, we summarize and discuss possible treatment options and their risks and benefits. Surgery should be performed soon after establishment of the diagnosis, which per se is often delayed despite a long-standing presence of attributable symptoms or signs in most cases. Following multilevel laminotomy, excellent results can be achieved by electrophysiologically guided microsurgical tumor removal in a single-staged or multistaged approach. The surgical goal is resection as gross total as possible provided intraoperative monitoring indicates preservation of function. Small tumor remnants often remain stable in the due course. In case of unresectable regrowth or recurrence, chemotherapy or radiotherapy are the adjuvant treatment options.