Familial Mediterranean fever (FMF) is a hereditary disorder characterised by recurrent attacks of fever with peritonitis or pleuritis, arthritis, myalgia or erysipelas-like skin lesions. The continuous inflammation in FMF is associated with increased serum amyloid A (SAA) protein which may lead to secondary amyloidosis and deposition of this insoluble protein in the kidney, gut, spleen, liver, heart etc. Therefore, treatment of patients with FMF is beneficial not only for the prevention of the acute attacks but also for improving their prognosis. In the present review we summarise the medical literature concerning FMF treatment, including new therapeutic agents and management of colchicine-resistant patients. Three electronic databases (MEDLINE, EMBASE, and the Cochrane Library) were searched from 1 January 1960 to 28 February 2010 for any therapeutic approach to FMF, with MeSH headings and text words (Familial Mediterranean Fever, FMF treatment, colchicine, infliximab, anakinra, SSRI). In conclusion, colchicine remains the mainstay therapeutic option in FMF. It is effective in various manifestations of the disease such as fever, peritonitis and pleuritis. It prevents the development of amyloidosis. It is safe in humans regarding fertility, and can be used during pregnancy and nursing. Dose adjustment should be made in patients with renal or hepatic failure. It is less effective in arthritis or myalgia, requiring additional treatment with NSAIDs and steroids. In the few cases where FMF is resistant to colchicine other measures, including corticosteroids, non-biological and biological DMARDs, interferon alpha and SSRIs should be employed.