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Abstract Background Idiopathic intracranial hypertension (IIH) is a disease entity characterized by elevated intracranial pressure, which usually accompanied by papilledema. However, diagnosing papilledema can be challenging in patients with preexisting ocular conditions, such as high myopia. Case presentation A 39-year-old woman with a long-standing history of high myopia presented with visual field constriction. Her visual acuity was decreased in both eyes, and she also reported morning headaches, pulsatile tinnitus, and intermittent periocular pain. Although she was diagnosed with IIH based on a cerebrospinal fluid study, there was no evident papilledema, which may have been masked by myopic changes in optic nerve and peripapillary retinal nerve fiber layer. After treatment for IIH with acetazolamide, her visual field and visual acuity markedly improved, and her symptoms were relieved. Conclusions In highly myopic patients, myopic changes in optic nerve head may obscure papilledema, complicating the diagnosis of IIH. This unusual case highlights the need for detailed history taking and a comprehensive clinical evaluation in patients with high myopia. Clinicians should suspect IIH in the presence of symptoms such as pulsatile tinnitus, morning headache, and visual field constriction, even in the absence of evident papilledema.