Abstract: Familial Mediterranean fever (FMF) is a kind of genetic autoinflammatory diseases, characterized with periodic fever, abdominal pain, arthritis and serositis. FMF is commonly seen in Mediterranean area and the onset of disease is mostly before 20 years old. Here we report a Chinese FMF patient who presented with typical syndromes after adulthood. The clinical manifestations, laboratory results, genetic mutations and therapeutic responses are presented and discussed in detail.