This study retrospectively analyzed brain magnetic resonance imaging (MRI) features in 17 patients with 1-2-hydroxyglutaric aciduria (1-2-HG), a rare inherited metabolic disease typically observed in children. The patients, aged 3 to 28 years, underwent MRI examinations, including proton MR spectroscopy in five cases. MRI findings revealed widespread white matter (WM) abnormalities, particularly in the subcortical WM, with the most severe involvement in the frontal regions. Subcortical abnormalities were the most common, followed by central and periventricular WM. The dentate nucleus was consistently affected, while the corpus callosum and cerebellar WM were spared. Cerebellar atrophy was observed in 11.8% of patients, and cystic changes were noted in two cases. Gray matter structures were involved in only one case. Monovoxel spectroscopy revealed decreased NAA and choline peaks in four patients. The study highlights the characteristic MRI features of 1-2-HG, which are consistent with previous literature, but also suggests that further studies with larger patient populations are needed to enhance the understanding and management of this rare disease.This study retrospectively analyzed brain magnetic resonance imaging (MRI) features in 17 patients with 1-2-hydroxyglutaric aciduria (1-2-HG), a rare inherited metabolic disease typically observed in children. The patients, aged 3 to 28 years, underwent MRI examinations, including proton MR spectroscopy in five cases. MRI findings revealed widespread white matter (WM) abnormalities, particularly in the subcortical WM, with the most severe involvement in the frontal regions. Subcortical abnormalities were the most common, followed by central and periventricular WM. The dentate nucleus was consistently affected, while the corpus callosum and cerebellar WM were spared. Cerebellar atrophy was observed in 11.8% of patients, and cystic changes were noted in two cases. Gray matter structures were involved in only one case. Monovoxel spectroscopy revealed decreased NAA and choline peaks in four patients. The study highlights the characteristic MRI features of 1-2-HG, which are consistent with previous literature, but also suggests that further studies with larger patient populations are needed to enhance the understanding and management of this rare disease.