Purpose: In paediatrics, Idiopathic Intracranial Hypertension (IIH) presents differently in pre-and post-pubertal children. This case report aims to bring awareness to uncommon presentations of IIH in a pre-pubertal child, highlighting the variability and exceptions of an atypical condition.Observations: A 7-year-old female with a BMI in the 84 th percentile presented to Rockhampton Base Hospital (Queensland, Australia) with a 2-month history of intermittent blurry vision, headache, vomiting, bi-temporal hemianopia and bilateral swollen discs. There were no common findings of obesity, neck stiffness, strabismus, abducens nerve palsy or common field defects such as enlarged blind spots, peripheral constrictions or paracentral scotomas. MRI brain/orbits with contrast performed had no MRI features of IIH, and lumbar puncture revealed an opening pressure of 6 cm H 2 0 with normal cerebrospinal fluid composition. Ophthalmologist, Paediatricians and Neurologist at Rockhampton Base Hospital and Lady Cilento Children's Hospital Brisbane (Queensland, Australia) were involved early on in the management of this patient. The patient's retinal nerve fibre layer thickness and field defects were found to be improving on formal HD-OCT and Humphrey Visual Field tests following early medical treatment with oral acetazolamide. Conclusion:This report is important to increase awareness of uncommon presentations of an atypical condition and encourage early involvement of multidisciplinary teams in the management and treatment of early adolescent children with this condition.