Only the minority come down with the illness during their puberty or even after reaching adulthood.[2,4] In the cases registered by the International Histiocyte Society, only 5% of patients are attacked after they are 5 years old.[2] This study reports a female child patient who presented with late-onset and recurrent hemophagocytic lymphohistiocytosis (HLH) arising from a new homozygous mutation of STX11; the relevant literature is reviewed. Here, STX11 is linked to hemophagocytic syndrome.