Malignant histiocytosis(MH)-like hemophagocytic syndrome(HS) is a fatal complication of nasal angiocentric lymphoma(AL) and difficult to distinguish from MH. Ten of total 42 patients with nasal AL had HS and 9 of them were initially suspected to have MH. Five patients had HS as initial manifestation, 3 at the time of relapse, and 2 during the clinical remission of lymphoma. Four patients were treated by combination chemotherapy(CHOP) and others had only supportive care. Immunohistochemical study and in situ hybridization were performed on the specimen obtained from 10 patients. The median survival of all patients from HS was 18 days(range 2 - 44 days) and all had fatal outcome regardless of the treatment-modality. All cases were positive for UCHL1(CD45RO) and Epstein-Barr virus (EBV) by EBER in situ hybridization. MH-like HS is a fatal complication of nasal AL and has a high association with EBV. Reactivation of EBV may contribute to HS and further investigation of predictive factors and effective treatment of HS should be pursued in future.