Familial Mediterranean Fever (FMF) is a rare hereditary auto-inflammatory disease that can be exceptionally associated with many other dys-immune disorders; the most reported associations were with systemic vasculitis, spondyloarthropathies, inflammatory bowel diseases, systemic lupus erythematous, multiple sclerosis, and juvenile chronic arthritis. The association of FMF with primary inflammatory myopathy remains exceptional and unusual; it has only been noted once before with adult polymyositis. We report an original observation of FMF associated with juvenile dermatomyositis in an eight-year-old boy, which, to our knowledge, has not been reported previously.