Sjögren's syndrome (SS) is a systemic lymphoproliferative, autoimmune disease, which is characterized by dryness of the eyes, mouth, and other mucous membranes. The nervous system may be affected in up to 20% of the cases of primary or secondary SS. We present a case of a 54-year-old woman with trochlear nerve palsy complicating Sjögren's syndrome secondary to rheumatoid arthritis. We suggest that all patients with multiple cranial neuropathies, especially when associated with rheumatoid arthritis, should be carefully examined for the possible presence of secondary SS.