Primary Sjogren’s Syndrome (pSS) is a chronic autoimmune disease with a variety of clinical presentations; however, isolated cranial nerve involvement has been described as a very rare presentation of pSS.

Our patient did not have any sicca symptoms, her symptoms were unilateral facial weakness (House–Brackmann score V), right-sided hearing loss and tinnitus. Initial work up included magnetic resonance imaging of the brain, serum testing for HIV, syphilis, varicella, ANA, anti-SSA, and anti SSB and CSF analysis.

MRI of the brain showed asymmetrical enhancement of the right facial nerve, serum studies showed positive ANA at 1:640 and positive anti-SSB and anti-SSA. The reminder of serum studies were negative. CSF showed lymphocytic pleocytosis, normal protein and glucose and no oligoclonal bands detected. Leading to the diagnosis of recurrent facial palsy due to Primary Sjogren’s Syndrome. She was previously treated with valacyclovir and oral prednisone with no improvement. The patient received two doses of IVIG and at 3 months follow-up, she reported improvement in facial weakness (House–Brackmann score III), hearing loss, and tinnitus.

In patient presenting with recurrent unilateral facial palsy, Sjogren’s Syndrome should be considered as an underlying cause of facial nerve mononeuropathy. Few cases have been reported in the literature with similar symptoms; however, our patient is unique in lacking preceding sicca symptoms and having concomitant hearing loss and tinnitus. Intravenous immunoglobulin should be considered in cases refractory to oral steroids.