Abstract: Histiocytic sarcoma is a rare kind of histiocytic lymphoma with an undetectable onset, no specific clinical and imaging mani-festations, and rapid progression. At diagnosis, the histiocytic sarcoma has already spread significantly in most patients. The diagnosis of histiocytic sarcoma depends on the basis of its mitotic figures and at least expression of one of the following: CD68, CD 163 , and lyso -zyme. Operation excision, radiotherapy, and chemotherapy are currently the more common treatment methods for histiocytic sarco -ma. However, no unified curative and treatment standard exists, and prognosis is poor. This review summarizes the diagnosis and treat-ment of histiocytic sarcoma.