Pseudotumor cerebri is a perplexing syndrome of increased intra-cranial pressure without a space-occupying lesion. The terminology for the disorder has changed over the years and the diagnostic criteria revised to reflect advances in diagnostic technology and insights into the disease process. The classification and nomenclature depend on the presence or absence of an underlying cause. When the diagnostic criteria are followed, a secondary etiology is unlikely. When no secondary cause is identified, the syndrome is termed "idiopathic intracranial hypertension."