With obscure etiology, confusing terminology, and controversial management, idiopathic intracranial hypertension (IIH), most commonly known as benign intracranial hypertension, remains a common and challenging clinical problem of the general and neurological practice. The authors present a case of a patient who was diagnosed with IIH and treated with serial lumbar punctures for 4 years prior to her final diagnosis of a large intracranial tumor. This case emphasizes that IIH, an enigmatic disease that affects young females and can potentially lead to blindness, should be diagnosed after an exhausting exclusion of secondary causes of intracranial hypertension.