© 2017 The American Laryngological, Rhinological and Otological Society, Inc. Objectives/Hypothesis: Spontaneous cerebrospinal fluid (CSF) leaks are associated with increased intracranial pressure (ICP) and considered a manifestation of idiopathic intracranial hypertension. Although postoperative acetazolamide and placement of CSF shunt systems are considered valuable interventions for elevated ICP, the impact on recurrence rate remains unclear. The objective of this study was to systematically review evidence from reported literature to evaluate whether postoperative ICP management reduces recurrence rates after primary endoscopic repair. Study Design: Prospective case series and systematic review. Methods: Demographics, defect location, success rates, and ICP management in spontaneous CSF leak patients were prospectively collected over 8 years. A search was also conducted in PubMed to identify studies reporting cases of spontaneous CSF rhinorrhea. Results: Fifty-six articles with nonduplicated data were identified and combined with a prospective series of 108 patients for a total of 679 patients treated for spontaneous CSF rhinorrhea. Average age was 50.4 years with 77% female. Average body mass index was 35.8 kg/m2. Defects were most commonly located in the sphenoid sinus (n = 334) followed by the ethmoid (n = 318) and the frontal sinus (n = 46). Successful primary repair was 92.82% in patient cohorts where ICP evaluation and intervention with acetazolamide or CSF shunt systems was performed, but was significantly decreased to 81.87% in series with no active management of elevated ICP (P <.001). Conclusions: Evaluation and intervention for elevated ICP in spontaneous CSF leaks is associated with significantly improved success rates following primary endoscopic repair. Level of Evidence: 4. Laryngoscope, 127:2011–2016, 2017.