This study describes the brain MRI features in 17 patients with L-2-hydroxyglutaric aciduria (L-2-HG aciduria), a rare inherited metabolic disorder. The patients, aged 3–28 years, underwent MRI scans, with five also undergoing proton MR spectroscopy. MRI findings included subcortical white matter (WM) abnormalities, cerebellar atrophy, and changes in the dentate nuclei and putamen. WM abnormalities were most severe in the frontal regions, with a symmetrical pattern. Subcortical abnormalities were the most common, followed by central and periventricular WM. The dentate nucleus was affected in all patients, while the putamen was involved in 71% of cases. The corpus callosum and cerebellar WM were spared in all patients. Gray matter involvement was minimal. Proton MR spectroscopy showed decreased NAA and choline peaks in four patients. The study found no significant correlation between disease duration and MRI severity, but white matter atrophy was related to disease duration. The findings support the diagnosis of L-2-HG aciduria with characteristic MRI features, including subcortical WM abnormalities, cerebellar atrophy, and dentate nucleus involvement. The study highlights the importance of MRI in diagnosing L-2-HG aciduria and understanding its progression. Limitations include the small sample size and retrospective nature of the study. Further research is needed to better understand the role of MRI in diagnosing and assessing the severity of L-2-HG aciduria.This study describes the brain MRI features in 17 patients with L-2-hydroxyglutaric aciduria (L-2-HG aciduria), a rare inherited metabolic disorder. The patients, aged 3–28 years, underwent MRI scans, with five also undergoing proton MR spectroscopy. MRI findings included subcortical white matter (WM) abnormalities, cerebellar atrophy, and changes in the dentate nuclei and putamen. WM abnormalities were most severe in the frontal regions, with a symmetrical pattern. Subcortical abnormalities were the most common, followed by central and periventricular WM. The dentate nucleus was affected in all patients, while the putamen was involved in 71% of cases. The corpus callosum and cerebellar WM were spared in all patients. Gray matter involvement was minimal. Proton MR spectroscopy showed decreased NAA and choline peaks in four patients. The study found no significant correlation between disease duration and MRI severity, but white matter atrophy was related to disease duration. The findings support the diagnosis of L-2-HG aciduria with characteristic MRI features, including subcortical WM abnormalities, cerebellar atrophy, and dentate nucleus involvement. The study highlights the importance of MRI in diagnosing L-2-HG aciduria and understanding its progression. Limitations include the small sample size and retrospective nature of the study. Further research is needed to better understand the role of MRI in diagnosing and assessing the severity of L-2-HG aciduria.