Familial Mediterranean fever (FMF) is an autosomal recessive disease o
f unknown etiology, characterized by recurrent self limited episodes o
f fever and polyserositis, Some patients develop generalized amyloidos
is, which can be fatal, Colchicine therapy modifies the natural histor
y of the disease by decreasing the attack frequency and preventing amy
loid deposition. The disease is common among Sephardic Jews, Arabs, Ar
menians and has also been sporadically found in other ethnic groups of
Mediterranean origin. We report two cases of FR IF in brothers living
in Abruzzo, Italy. They were born from consanguineous parents and com
plained typical symptoms since childhood, The boy suffered from one fe
brile attack every week; he presented three episodes of acute scrotum
at age 8 and 9, The elder sister showed a spontaneous partial relief d
uring adolescence, Juvenile rheumatoid arthritis was suspected and Asp
irin was used for many years without any clinical improvement, Treatme
nt with colchicine 1 mg/day was established at age 13 and 17 respectiv
ely, and a sudden reduction of frequency of attacks was obtained. A gi
ngival biopsy did not show amyloid, The three elder brothers are, at p
resent, in good health, Our experience point out the diagnostic diffic
ulties of FMF expecially in a country were the disease is uncommon.