Idiopathic intracranial hypertension (IIH) is characterized by symptoms and signs of elevated intracranial pressure, elevated cerebrospinal fluid (CSF) pressure, normal CSF content, and normal brain with normal or small ventricles on neuroimaging studies. IIH in children has a wide spectrum of clinical presentation. Diagnostic criteria with modifications to adapt to the variations in children are discussed. Diagnostic and therapeutic options are reviewed.