OBJECTIVES: Spontaneous intracranial hypotension (SIH) is a debilitating neurologic condition that is often thought of as separate from idiopathic intracranial hypertension (IIH). The unique case presented here details a spontaneous spinal cerebrospinal fluid (CSF) leak that developed abruptly following a CSF pressure elevating maneuver in a patient with pre-existing intracranial hypertension, raising the possibility of a causative link between the two conditions. RESULTS: A 40-year-old woman with symptomatic IIH developed a dural tear of a thoracic spinal nerve root sleeve during an episode of Valsalva maneuver leading to a CSF leak and development of symptomatic SIH. This was successfully treated with epidural blood and fibrin glue patch and the patient is now symptom-free. DISCUSSION: The implication of a possible causative link between elevated CSF pressure and subsequent development of dural rupture and SIH raises important questions regarding the pathophysiology of SIH in some cases. Furthermore, it suggests that there could be a potential prophylactic benefit of CSF pressure lowering medications in preventing the development of SIH in patients with IIH.