To report a case of papilledema and pseudotumor cerebri developed in association with Sjögren's syndrome.
Case-report of a 38-year-old woman with history, imaging and histology confirming the diagnosis of both pseudotumor cerebri and Sjögren's syndrome who presented with bilateral decrease of vision.
Papilledema associated with pseudotumor cerebri was observed in both eyes. The patient's visual acuity improved transiently with the administration of intravenous steroids and cyclophosphamide; subsequently she needed a ventriculoperitoneal shunt.
Sjögren's syndrome should be considered in the different etiologies of pseudotumor cerebri. The major improvement with corticosteroids and ventriculoperitoneal shunt makes prompt diagnosis essential.