Pseudotumor cerebri syndrome comprises intracranial hypertension with usual accompaniment of papilledema without ventriculomegaly, brain tumor, or brain dysfunction. Primary Sjögren's syndrome is a common autoimmune connective tissue disease that may present with neurologic symptoms as its first manifestations. While central nervous system involvement in primary Sjögren's syndrome is rare, we report a case with pseudotumor cerebri syndrome as the initial presenting symptoms.