Background: Central nervous system involvement with primary Sjögren's syndrome (pSS) is rare, and often undiagnosed. Sjögren's syndrome and concomitant intracranial hypertension is a very rare event.
Case: We describe a patient with pSS who presented with intracranial hypertension as the initial symptom of the disease.
Conclusion: This is the first case report of a patient with pSS that presented with intracranial hypertension only.