Diagnosis of FMF is primarily clinical and based on the use of the Tel-Hashomer diagnostic criteria, divided into major and minor signs, as shown in Table 2: the presence of two major criteria, or one major and two minor criteria, allows for a definitive FMF diagnosis, while the presence of a single major criterion and one minor one may point towards a probable diagnosis, which can be confirmed thereafter by the presence of mutations in the MEFV gene [40]. This evidence concerns the gene MEFV and familial Mediterranean fever.