Introduction: L-2-hydroxyglutaric aciduria (L-2-HGA) is a rare autosomal recessive neurometabolic disorder caused by pathogenic variants in L2HGDH, leading to accumulation of the neurotoxic metabolite L-2-hydroxyglutarate. It typically presents with progressive developmental delay , seizures, and characteristic but non-specific bilateral white-matter abnormalities on MRI. Diagnostic delay is common due to overlap with leukodystrophies and epileptic encephalopathies. Early-onset status epilepticus is an uncommon presentation. Case presentation: We report a 7-year-old boy with speech delay , learning difficulties,behavioural disturbances, and recurrent generalized tonic–clonic seizures. Notably , he experienced two episodes of status epilepticus in infancy . Neurological examination was normal. EEG demonstrated diffuse background slowing without epileptiform discharges. BrainMRI showed bilateral, symmetric confluent periventricular, deep, and subcortical white-matter signal abnormalities with relative U-fiber sparing, initially suggestive of leukodystrophy . Urine organic acid analysis revealed marked isolated elevation of 2-hydroxyglutaric acid, with only a trace increase in ethylmalonic acid. Whole-exome sequencing identified a pathogenic L2HGDHvariant, confirming L-2-HGA. Other metabolic and systemic investigations were unremarkable. Riboflavin and L-carnitine supplementation were initiated. Conclusions: This case expands the recognised clinical spectrum of L-2-HGA by highlighting early-onset status epilepticus with leukodystrophy-like MRI changes. Neuroimaging alone may be misleading; integrated metabolic and molecular evaluation is essential for accurate diagnosis. Increased awareness of this atypical presentation may facilitate earlier recognition,targeted investigation, appropriate counselling, and timely management, particularly in resource-limited settings.