Background: Histiocytic sarcoma (HS) is a rare hematolymphoid neoplasms whose cells show morphologic and immunophenotypic features of mature tissue histiocytes. We herein report a HS case without nodules or lymphadenectasis confirmed with the help of immunohistochemistry (IHC) on bone marrow (BM) biopsy and smear.Case presentation: A 63-year-old female patient with a history of cerebral infarction presented with fever, retching and hypodynamic sign for three weeks. The peripheral blood examination showed aggressive pancytopenia accompanying with the positivity for Epstein-Barr virus (EBV) antibody. The computed tomography and abdomen ultrasound scan didn’t reveal any nodules or lymphadenectasis other than hypersplenotrophy. Significantly, the BM aspirate shows vast pleomorphic tumor cells atypically distributed in both forms of single diffuse and cohesive. The hemophagocyte phagocytized granulocytes on BM smear exhibited the lymphoma related hemophagocytic syndrome. Immunohistochemically, neoplastic cells were immunopositively for macrophage-associated antigen cluster of CD4, CD68, CD163, but negative for the T-cell, B-cell and myeloid lineage markers of CD15, CD20, PAX-5, CD5, CD30, CD3, CD56, CD38, CD138, ALK and MPO, confirming the hypothesis of HS in BM. Conclusion: The rare HS case of bone marrow with atypically partial cohesive pleomorphic tumor cells had the hemophagocytic syndrome, presented highly aggressive clinical course and challenged to the diagnoses.