Optic nerve sheath(ONS) enhancement in idiopathic intracranial hypertension (IIH) patients has been reported in recent years. In this retrospective observation, we analyzed the clinical characteristics of IIH patients with enhancement of ONS.Eighty-two patients with clinically diagnosed IIH from January 2017 to December 2019 were under observation. Then, based on the presence of contrast-enhancement (CE) in ONS on orbital magnetic resonance image (MRI), the IIH patients were divided into CE-ONS group and no-CE(NCE)-ONS group. Six months follow-up information was also included in the observation study. By comparing clinical data of the two groups of IIH patients, we tried to evaluate whether there is clinical heterogeneity in CE-ONS patients.12 patients were included in CE-ONS group, 10 females and 2 males. 70 patients were included in NCE-ONS group, 56 women and 14 men. We found that patients with CE-ONS had a longer course of disease (median disease duration before diagnosis, 5 months vs. 3months, P<0.01) and more likely had the sign of distension of the perioptic subarachnoid space (DPSS) (58.33 % vs. 24.29 %, P = 0.034). But no significant differences were found in demographic characteristics, clinical symptoms, degree of visual impairment, papilledema, opening pressure(OP) on lumbar puncture and clinical outcomes.As a rare sign on MRI, ONS enhancement can occur in patients with IIH. IIH patients with CE-ONS may have a longer course of disease and more prone to DPSS, but there is no significant difference in clinical manifestations, OP, and clinical outcomes compared with IIH patients without CE-ONS.Copyright © 2023 The Authors. Published by Elsevier B.V. All rights reserved.