Idiopathic intracranial hypertension (IIH) is a condition characterised by increased intracranial pressure of unknown cause predominantly seen in obese women of childbearing age and associated with a history of recent weight gain. The aetiology is poorly understood and there are no evidence-based guidelines on the management of the disease. We aim to provide a review of the recent literature outlining the latest advances in this field over the past few years. Areas of emerging interest related to the pathophysiology of IIH will be discussed, such as the role of obesity, adipose tissue and 11β-hydroxysteroid dehydrogenase type 1. We consider the latest research on the role of venous sinus stenosis in IIH and ex vivo advances into cerebrospinal fluid drainage via the arachnoid granulation tissue. The latest techniques for optic nerve head evaluation and the role of optical coherence tomography will be summarised. Finally, we will discuss recent advances in the management of IIH, including weight loss, and medical and surgical treatment strategies.