Abstract: Hemophagocytic lymphohistiocytosis (HLH) is a fatal clinical syndrome which is caused
by dysregulated immune response. It is mainly characterized by fever, pancytopenia, splenomegaly
and hemophagocytosis in bone marrow or other tissues. The diagnosis of HLH depends on clinical
signs and laboratory findings. This article reported a case of HLH first presenting as liver injury
during pregnancy who were successfully treated with steroids and gamma-globulin.