Familial mediterranean fever (FMF) is a disease whose importance in recent years is reappearing thanks to the advances in molecular genetics. The diagnosis is established by symptoms, presence of inflammatory episodes of fever and serositis, family background and genetic study. Identification of the most prevalent mutations of the MEFV gene may confirm atypical or incomplete forms of FMS that are difficult to recognize based on the classical major and minor criteria. Knowledge of this more extensive clinical spectrum makes it possible to have a new diagnostic and therapeutic perspective based on the treatment of colchicine and secondary prevention of AA amyloidosis.