The authors present their experience with the operative management of 20 intra-axial tumors of the cervicomedullary junction. There were two distinct modes of clinical presentation: lower cranial nerve dysfunction and spinal cord dysfunction. Both groups of patients had indolent courses: in 75% of the patients the symptoms had been present for 6 months to 2 years. Radical excision was carried out in all patients. There was no surgical mortality. Postoperative neurological recovery was directly related to the preoperative status; one patient had a significant new deficit. The authors conclude that intrinsic gliomas of the cervicomedullary junction are amenable to radical excision and that an aggressive surgical approach offers the potential for both neurological recovery and long-term survival. The neuroradiological evaluation and operative technique are discussed.