Idiopathic intracranial hypertension (IIH), or pseudotumor cerebri, is a condition of elevated intracranial pressure in the absence of clinical, laboratory or radiological evidence of an intracranial space occupying lesion that can occur in the pediatric population. While 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. This review discusses the demographics, clinical presentation, diagnosis, neuroimaging, and management of pediatric IIH. Common etiologies of IIH in children, with particular focus on endocrine abnormalities, are highlighted.

Idiopathic intracranial hypertension (IIH), also known as primary pseudotumor cerebri syndrome (PTCS), is a condition of unknown etiology which affects primarily overweight, reproductive-aged women and causes increased intracranial pressure (ICP). This review discusses the recently revised diagnostic criteria for PTCS for adults and children. Additionally, the role of obesity in the epidemiology, etiology, and management of IIH as well as the current knowledge of obesity profiles and markers in IIH are reviewed. We also highlight the emerging, unifying theory of the neuroendocrine effects on the mineralocorticoid receptor to explain a possible mechanism for the increased cerebrospinal fluid production and ICP in secondary PTCS.