PURPOSE OF REVIEW: Cerebrospinal fluid leaks that occur spontaneously are challenging to manage clinically owing to frequent recurrences following attempted surgical closure. Understanding of the underlying pathophysiology has increased in recent years as we now recognize that the vast majority of these patients demonstrate clinical symptoms and radiographic signs of elevated intracranial pressure. Individuals with this disorder also arise from a distinct demographic group. Increased knowledge of the characteristics of this patient population along with updated medical and surgical management will provide increased success rates in the management of this clinical entity. RECENT FINDINGS: Current literature indicates that control of intracranial hyptertension, coupled with endoscopic repair, will improve success rates comparable with other etiologies. Recent information on the underlying pathophysiology of spontaneous cerebrospinal fluid leaks is discussed. Improvement in preoperative identification of radiographic signs of intracranial hypertension (i.e. empty sella), operative technique, and postoperative management of elevated intracranial pressure are also reviewed. SUMMARY: We highlight the demographic characteristics, clinical presentation, radiologic findings, and clinical management of patients with this disorder. © 2009 Lippincott Williams & Wilkins, Inc.