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While idiopathic intracranial hypertension (IIH) is more commonly recognized as a disorder of adults, it affects children of all ages and can have distinctive characteristics when presenting in the prepubertal age group. It is characterized by raised intracranial pressure (ICP) in the absence of brain parenchymal lesion, vascular malformations, hydrocephalus, or central nervous system (CNS) infection. The diagnosis is usually confirmed by a high opening pressure of cerebrospinal fluid (CSF) with exclusion of secondary causes of intracranial hypertension. Objectives: The purpose of this review is to summarize relevant articles on the diagnostic tools used in evaluation and management of pediatric IIH. A summary of demographics, clinical presentation, diagnosis, neuroimaging, as well as existing evidence of treatment strategies is presented. Method: We conducted a bibliographic search in PubMed using the terms idiopathic intracranial hypertension (IIH), Pseudotumor Cerebri syndrome (PTCS). The review of the literature revealed a lack of published consistent data on the diagnosis criteria and management of idiopathic intracranial hypertension in children. Conclusion:This article provides a review of IIH in children and revised diagnostic criteria based on recent evidence and published opinion. Relative to the adult population, the demographic features and clinical presentation of IIH as well as the diagnosis and treatment guidelines for children are quite different. This review highlights the importance of early recognition and management of IIH to prevent permanent visual loss. Key words: idiopathic intracranial hypertension, pseudotumor cerebri syndrome Idiopathic intracranial hypertension (IIH), also referred to as Pseudotumor Cerebri, is characterized by elevated intracranial pressure without discernable etiology, with normal cerebrospinal fluid content, and normal contrast-enhanced computed tomography (CT) or magnetic resonance irradiation (1,2,3).

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