Oligodendrogliomas (OD) are rare, diffusely infiltrating tumors, arising in the white matter of cerebral hemispheres, and displaying better sensitivity to treatment and prognosis than other gliomas. Favorable prognostic factors are low-grade, combined loss of 1p/19q, younger age, good performance status, and frontal localization. Low-grade OD usually present with seizures, whereas high-grade tumors often present with focal deficits, increased intracranial pressure or cognitive deficits. Treatment may be deferred until progression in young patients with low-grade OD presenting with seizures only. Patients with enhancing lesions, mass effect, focal deficits or increased intracranial pressure should be treated without delay. Treatment consists of resection as extensive and as safe possible. Postoperative radiotherapy is indicated for large, unresectable, or incompletely resected tumors; focal deficits; anaplastic tumors; or enhancing lesions. Adjuvant PCV chemotherapy increased progression-free survival but does not improve survival as compared to PCV given at recurrence. Chemotherapy with either PCV or temozolomide constitutes a standard for recurrent/progressive disease.