Oligodendroglioma is the third most common glial neoplasm and most commonly arises in the frontal lobe. It occurs in males more frequently, and the peak manifestation is during the 5th and 6th decades. Children are affected much less commonly. The clinical presentation is often of several years duration with most patients presenting with seizures, reflecting the strong predilection of this tumor to involve the cortical gray matter. Current histopathologic classification schemes recognize two main types of tumors: well-differentiated oligodendroglioma and its anaplastic variant. Less commonly, neoplastic mixtures of both oligodendroglial and astrocytic components occur and are termed oligoastrocytomas, with both well-differentiated and anaplastic forms. Surgical resection is the mainstay of initial treatment, and many patients experience a long progression-free period. Recent genotyping has revealed chromosomal loss of 1p and 19q as a genetic signature in most oligodendrogliomas, and these tumors respond favorably to chemotherapy. Hence, radiation therapy is now generally reserved for partially resected tumors and cases that failed to benefit from chemotherapy. At cross-sectional imaging, the tumor characteristically involves the cortical gray matter and frequently contains calcification. Robust enhancement is not a common feature and suggests transformation to a higher histologic grade. Advanced magnetic resonance imaging techniques and metabolic imaging play increasingly important roles in both pre- and postoperative assessment of these complex neoplasms.