Familial Mediterranean fever (FMF) is the most common autoinflammatory disease characterized by recurrent self-limited attacks of fever, accompanied with peritonitis, pleuritis or arthritis. It is well known that FMF may coexist with vasculitic pathologies, especially with those involving small and medium vessels. Among the vasculitic pathologies reported to be associated with FMF, Henoch-Schonlein purpura and polyarteritis nodosa come the first, possibly followed up by protracted febrile myalgia. However, coexistence of FMF with any large vessel vasculitis has not been reported to date. Here, we present a case with FMF who later developed Takayasu arteritis, with a severe disease course, being resistant to corticosteroids and conventional immunosuppressive agents, and requiring infliximab treatment.