Idiopathic intracranial hypertension (IIH) is a disease of unknown etiology associated with increased intracranial pressure, predominantly affecting obese females of childbearing age. The history of IIH is quite unique, featuring only limited advancements in evidenced-based treatments, but boasting literally countless changes in nomenclature, proposed etiology, and conceptual approach. Despite its elusive pathogenesis, an evolution of our approach to IIH can be traced sequentially through identifiable periods. Contemporary research suggests that we are approaching a new phase in IIH, redefining it as a global neurologic syndrome with more far-reaching effects than previously realized.