We present a 55-year-old woman with periodic fever and symptoms similar to adult-onset Still's disease (AOSD). She had a heterogeneous mutation of the MEFV gene and colchicine was effective. Atypical familial Mediterranean fever (pyrin-associated autoinflammatory disease) should be considered in patients with periodic fever accompanied by symptoms similar to AOSD.
Keywords: Familial Mediterranean fever; adult‐onset Still’s disease; elevated liver enzymes; pyrin‐associated autoinflammatory diseases; skin rash.