PURPOSE. A 32-year-old Saudi female presented with typical Kikuchi-Fujimoto disease, i.e., fever, cervical lymphadenitis and leukopenia, but there was also painful upper eyelid swelling with pain on upgaze. METHODS. A connective tissue disease and lymphoma workup were unremarkable, as were antibody titers to Apifia felis and Bartonella henselae. RESULTS. Orbital computed tomography showed significant lacrimal gland enlargement. Cervical node biopsy revealed necrotizing lymphadenitis. CONCLUSION. Concomitant lacrimal gland inflammation and cervical lymphadenopathy may be a benign self-limited disease.