Autoimmune lymphoproliferative syndrome (ALPS) is a rare inherited disorder of apoptosis, most commonly due to mutations in the FAS (TNFRSF6) gene. ALPS caused by defective lymphocyte homeostasis is characterized by non-malignant lymphoproliferation that often improves with age and is an autoimmune disease, mostly directed toward blood cells. This report describes a 17-year-old female with ALPS who developed skin rashes and aphthous stomatitis after using colchicine therapy owing to Familial Mediterranean Fever (FMF) with V726A heterozygous mutation in MEFV gene, hepatosplenomegaly, lymphadenopathy and pancytopenia, elevated vitamin B 12 and IL-10, elevated double-negative T cells (DNTs) and elevated immunoglobulin (Ig) G, consistent with a heterozygous germline FAS mutation [p.E261K (c.781G>A)]. In our country where genetic diseases are common due to consanguineous marriages, diseases with serious morbidity such as ALPS should be kept in mind. We should not forget that autoinflammatory diseases and familial Mediterranean fever can coexist owing to very high carrier rate in our country.