Although no single variant in an AID‐associated gene seemed frequent in FMF cases, we found that six different variants in four known genes (PRF1, STXBP2, RAB27A and UNC13D) associated with familial haemophagocytic lymphohistiocytosis (FHL) were present in about 20% of our FMF patients. The gene discussed is STXBP2; the disease is hemophagocytic syndrome.