OBJECTIVE The goal in this study was to review a series of patients who underwent surgical removal of intramedullary high-grade gliomas, focusing on the functional outcome, recurrence rates, and technical problems continually debated in neurosurgical practice. METHODS Between December 1976 and December 2006, 22 patients underwent removal of intramedullary high-grade gliomas. Lesions were located in the cervical spinal cord in 12 patients, and in the thoracic cord in 10. RESULTS Histological examinations showed 10 Grade III astrocytomas and 12 glioblastomas. Only 2 of the 22 high-grade astrocytomas could be completely removed. The clinical postoperative status worsened in 14 patients (63.6%), was unchanged in 7 patients (31.8%), and there was 1 case of intraoperative death (4.5%). None of the 22 patients showed improvement in their neurological status postoperatively. In this series, excluding the 1 intraoperative death, all patients died of progression of the malignancy. CONCLUSIONS Surgical treatment did not ameliorate the postoperative neurological status; instead, in the majority of cases, it prompted a worsening of the deficit. Radiotherapy and chemotherapy have a little influence on the length of survival. In this series, multimodality treatment of intramedullary high-grade astrocytomas has been shown to increase length of survival without improving the neurological status.