A 69-year-old Tunisian female was admitted in 2005 for arthritis and fever. The examination revealed a temperature of 39.7 C, an evanescent diffuse maculopapular rash and symmetrical polyarthritis involving knees, wrists and metacarpophalangeal joints. There was neither lymphadenopathy nor hepatosplenomegaly. Laboratory evaluation revealed: Hb: 74g/l, a white blood cell count of 17.6x109/l (neutrophils: 89%) platelets: 471x109/l, erythrocyte sedimentation rate of 145mm/h and C-reactive protein: 440mg/l. The liver function test scores were elevated: AST: 56 IU/l, ALT: 53IU/l (normal value: 10 to 40IU/l), GGT: 124IU/l (normal value: 10-35IU/l). The serum ferritin was elevated: 3000ng/ml (normal value: 11 to 250ng/ml). Urinalysis was normal and proteinuria was absent. Serological evaluation for hepatitis B and C, Epstein-Barr Virus (EBV), Cytomegalovirus, Herpes simplex, Chlamydia, Mycoplasma, Rickettsia conorii, Coxiella burnetti, Toxoplasma, Borrelia, Aspergillus, Candida, agglutination test for Brucella were negative. Blood, urine and stool cultures were also negative. Tuberculosis was discarded by acid-fast stained smears and culture. Laboratory evaluation for systemic or malignant diseases were negative (antinuclear antibodies, anti-DNA antibodies, antiphospholipid antibodies, anti mitochondrial antibodies, anti smooth-muscle antibodies, rheumatoid factor, Antineutrophil Cytoplasmic Antibodies (ANCA), cryoglobulins, tumor markers). Chest x-rays, ultrasound examination, and echocardiography were normal as well. The temporal artery biopsy and the bone marrow examination were normal. We diagnosed AOSD according to the Yamaguchi criteria and we started a steroid therapy (prednisone: 1mg/kg/day). The symptoms began to improve. Ten months later, she presented the same clinical picture. X-rays of both hands showed bone erosions at the carps. We used oral methotrexate in weekly dose of 10mg. 8 months later, the patient developed an ulcerated tumor at the scalp with cervical lymph nodes whose biopsy revealed a squamous cell carcinoma. Methotrexate was stopped and she was treated with chemotherapy and radiotherapy. She did not present a recurrence during a 12 months follow up period.