A 29-year-old woman had an isolated, pupil-sparing third cranial nerve palsy. Serologic and CSF abnormalities and the subsequent course were consistent with systemic lupus erythematosus. Corticosteroid therapy resulted in improvement of ocular palsy within 4 weeks. The pathogenesis of cranial neuropathy in systemic lupus erythematosus and the unique presentation in this patient are discussed.