A 2-year-old girl who was under genetic follow up for developmental delay and dysmorphism was accidentally found to have bilateral optic disc swelling during screening examination. She showed response to optokinetic drum examination and the anterior segment examination was unremarkable. Optic disc swellings were seen in both eyes. Lumbar puncture shows high opening pressure of 50 cm H2O with unremarkable CSF analysis. MRI of brain was done and showed features in keeping with mild cerebral atrophy, with no evidence of hydrocephalus or space occupying lesion. She was diagnosed with idiopathic intracranial hypertension and oral acetazolamide 125mg bd was commenced. However, papilloedema persist despite medical therapy. Ventriculoperitonel shunt was inserted to reduce the csf pressure. This case report highlights the importance of considering idiopathic intracranial hypertension as a cause of optic disc swelling in pre-pubertal children because delay in diagnosis and treatment may permanently affect visual function especially in children.