Idiopathic intracranial hypertension (IIH) is a syndrome caused by increased intracranial pressure without any evident cause, previously known as pseudotumor cerebri. It is a condition with a predominance in obese women of reproductive age and is less often seen in men and children. The pathophysiology of IIH is not well understood, and several mechanisms are suggested to play a role, such as excessive cerebrospinal fluid (CSF) production, impaired CSF absorption, increased intravascular volume, increased intracranial venous pressure, and, most recently, alterations in the glymphatic system. Known risk factors are certain medications, hormonal alterations, infections, and autoimmune disorders. Main clinical symptoms of IIH are headache and vision loss, less frequently pulsatile tinnitus, diplopia, photopsia, eye pain, or cranial nerve palsies. Ophthalmological evaluation usually reveals papilledema. Lumbar puncture demonstrates elevated opening CSF pressure. The role of clinical neuroradiology is firstly to exclude causes of secondary intracranial hypertension due to space-occupying masses, hydrocephalus, infection, or vascular pathologies, including venous sinus thrombosis. Secondly, imaging can show findings that strongly support the diagnosis of IIH such as enlargement of the perioptic CSF spaces, optic nerve tortuosity, flattening of the posterior aspects of the globes, intraocular protrusions of the optic nerve heads and their contrast enhancement, partially empty sella, and transverse sinus narrowing, followed by less specific signs such as enlarged Meckel’s cave, small meningoceles within the skull base, slit-like ventricles, or acquired tonsillar ectopia. Recommended radiological protocol includes contrast-enhanced MR examination of the brain and orbits, followed by MR venography.