In 30 p. 100 of Kikuchi's disease (or necrotizing histiocytic lymphadenitis), polymorphous and non specific cutaneous manifestations are present. We report herein an original case of Kikuchi's disease in which eyelid edema was the first sign.An 18-year-old girl was referred to our department for fever, arthralgia, cervical lymphadenitis and an important eyelid edema which had begun 4 days before. The rest of physical examination was normal. Laboratory tests revealed pancytopenia, elevated sedimentation rate, increased transaminases and normal muscular and thyroid tests. Various serologic studies were also negative. Thoracic CT scan, abdominal ultrasound and bone marrow biopsy showed no sign of lymphoma. Cervical lymph node biopsy revealed necrotizing histiocytic lymphadenitis, without neutrophils, suggesting the diagnosis of Kikuchi's disease. Eyelid edema due to lacrimal gland inflammation was resolved after local injections of cortisone. Our patient recovered without therapy within 3 weeks. No recurrence was observed after 4 months.Kikuchi's disease is rare and benign. It is clinically manifested by cervical or generalized lymphadenopathy, with fever. Diagnosis is made by lymph node biopsy showing necrotizing histiocytic lymphadenitis. The etiology is not yet well known, although a viral cause is often suspected. The main differential diagnoses of Kikuchi's disease are lupus erythematosus and lymphoma. Skin lesions are not well described. To our knowledge, we report herein the first case of eyelid edema revealing Kikuchi's disease. Therefore, Kikuchi's disease should now be considered as a new cause of eyelid edema.