There have been only case reports concerning the management of intramedullary spinal cord gangliogliomas. We review our experience of 56 patients with respect to functional status, progression-free survival and long-term outcome. In this retrospective review, 56 patients, 35 males and 21 females ranging in age from 7 months to 25 years (mean, 7.0 y), underwent surgical treatment for intramedullary gangliogliomas located throughout the spinal cord. Forty-six patients had gross total resection as confirmed by postoperative imaging studies, and 10 had radical subtotal resection. Only five patients underwent postoperative irradiation or chemotherapy after surgery at our center and therefore outcome was attributed to surgery alone. There were no operative deaths, and the 5-year actuarial survival rate was 88%. The progression-free survival rate at 5 years was 67%. Neurological function in the surviving patients at recent follow-up evaluation was stable or improved in 72%. Patients have a long survival following radical surgery. These tumors have an indolent course, and radiotherapy appears to be unnecessary.