Clinical symptoms and findings of familial Mediterranean fever (FMF), occur as a result of autoimmune inflammation of the serous membrane which is also seen in systemic lupus erythematosus (SLE). Difficulties are sometimes encountered in the differential diagnosis of FMF because of similar clinical features with other autoimmune inflammatory diseases, and very rarely it can be seen with SLE. The association of FMF and SLE has been reported in one childhood case in Turkey. As far as we know, there is no report in the adult age group. Here, we present the first FMF and SLE association in an adult and discuss the pertinent literature in Turkey.