Familial Mediterranean fever (FMF) is an inherited disease characterized by recurrent attacks of fever and accompanying peritonitis, pleuritis, arthritis or erysipelas-like skin disease. The relationship between FMF and spondyloarthropathy (SpA) remains controversial. We described a particular case of 36-yr-old male patient with FMF and ankylosing spondylitis with negative HLA-B27. This case had late-onset FMF in spite of homozygote M694V mutation and his typical acute attacks of FMF began many years after the initiation of complaints related to sacroiliitis. There is no general consensus on whether the association of FMF and SpA is only an occasional coincidence or they are clinically and pathologically linked two conditions. Further studies with larger series are required to clarify the relationship between FMF and SpA
