AA amyloidosis may be a complication of Familial Mediterranean Fever (FMF). This 
is a case history of a female patient who did not have the classic symptoms of 
FMF, which usually precede the renal manifestation. The patient was admitted 
with edema of both legs, and the nephrotic syndrome was discovered, leading to 
the diagnosis of AA amyloidosis on kidney biopsy. Genetic testing uncovered the 
homozygous M694V type mutation, the most common mutation of FMF, which renders 
the patients prone to amyloidosis. This case represents the phenotype II of FMF, 
which presents with amyloidosis without prior classic attacks of FMF. Since 
effective prevention of the development of amyloidosis is available, genetic 
testing should be considered in order to identify mutations which carry high 
risk for the development of amyloidosis. This is also relevant in asymptomatic 
individuals with family history of FMF.