Objectives: This pilot selective study aimed to explore relationship between intracranial hypertension (IIH) and development of pulsatile tinnitus (PT) and the impact of its treatment on severity of PT. Patients & Methods: All patients presenting to ENT clinic with PT were evaluated and only patients fulfilling Modified Dandy's Criteria for the diagnosis of IIH were enrolled in the study. Patients underwent full otorhinolaryngological exmaination and tinnitus was graded using the Tinnitus Handicap Inventory (THI). Severity of headache was assessed using the 11-point Numeric Rating Scale (NRS) and detailed ophthalmic examination was performed. All patients underwent lumber puncture (LP) and opening pressure was measured, then patients were maintained on acetazolamide 1–2 g/day. If patients did not show improvement or if the condition recurred after initial improvement, patients underwent insertion of lumbo-peritoneal shunt (LPS) for permanent drainage. Results: Six PT patients had IIH; 5 females and one male with mean age of 31.5±6.2 years and mean BMI of 35.1±3.5 kg/m2. All patients had PT that completely disappeared on jugular compression with mean THI score of 22±13.3. At time of discharge, all patients showed improved headache, tinnitus completely disappeared in five patients, while the 6th patient had THI grade 1 with a mean total THI score of 8±6.9 with significant difference versus at admission score. Throughout mean follow-up period of 15.5±3.9 months; one patient showed recurrence of headache and tinnitus and underwent LPS insertion for permanent CSF drainage and patient reported complete relief of her symptoms and tinnitus disappeared completely. Conclusion: There is close association between IIH and PT. IIH-directed therapy provided nearly complete relief of tinnitus with resolution of other manifestations. Presence of PT must arouse suspicious for being secondary to increased ICP and could be used as prognostic sign for assessing treatment efficacy and follow-up for the possibility of recurrence.