L-2 hydroxyglutaric aciduria (L2HGA) is an inherited neurometabolic disease characterized by elevated levels of L-2 hydroxyglutaric acid in urine, plasma, and cerebrospinal fluid. The disease has a progressive clinical course presenting with ataxia, seizure, and psychomotor retardation. Magnetic resonance imaging (MRI) is helpful to make the diagnosis by providing specific findings, such as subcortical white matter, symmetrical globi pallidi, and dentate nuclei hyperintensities, on T2-weighted images. Another important role of MRI is to detect associated intracranial malignancies in patients with L2HGA. The association between L2HGA and cerebral neoplasms has not been clearly elucidated to date; however, a few cases reports in the literature support this association. We report a patient with L2HGA associated with anaplastic oligodendroglioma and review the relevant literature.