Oligodendroglial tumors have historically been a fatal disease, despite maximum medical and surgical treatment. However, in the past 10 years, effective adjuvant therapies have been developed that have been found to significantly prolong patient survival and, in some instances, provide a cure. Improvements in our understanding of the molecular biology of these tumors, refinements in surgical technique, and advances in chemotherapeutic treatment have combined to produce this improvement in survival with little additional toxicity to patients. At the time of diagnosis, patients should undergo maximum surgical resection or needle biopsy if maximum surgical resection is not possible. Patients with low-grade lesions may be followed, receiving radiation only at the time of progression. Patients with high-grade lesions should be irradiated up front. In addition, selected patients should receive chemotherapy. Clinical trials remain critical to advancing our understanding of these tumors and improving our ability to cure these patients.