Primary Sjögren syndrome is a relatively common auto-immune disease. It may cause different neurological signs by the involvement of central and peripheric nervous systems.

Symptoms of mouth-eye dryness, oculomotor and facial nerve mononeuritis, positivity of Schirmer's test, decreased salivation, chronic lymphocytic sialoadenitis at salivatory gland biopsy and presence of anti-SSA (anti-Ro) were detected in a 74 year old female patient. The patient was diagnosed as Sjögren syndrome with the signs of cranial nerve neuropathy. Proptosis of left eye, increased retro-orbital fatty tissue, thickness of optic nerve sheath, bilateral periventricular and thalamic infarcts were detected at orbital and cranial magnetic resonance imaging.

Primary Sjögren syndrome should be considered in differential diagnosis and be investigated in cases that have idiopathic lesions of focal or diffuse central nervous system and peripheric nervous system.