The study was aimed to explore the diagnostic significance of hemophagocytosis in the patients with hemophagocytic lymphohistiocytosis (HLH). 61 suspected HLH patients from June 2005 to October 2008 were enrolled in the study. The suspected HLH patients were divided into confirmed group (43 out of 61) and excluded group (18 out of 61) according to HLH-2004 diagnostic criteria. The incidences of hemophagocytosis in bone marrow, spleen or lymph nodes were compared in all groups. The results indicated that the hemophagocytosis in bone marrow, spleen or lymph nodes was found in 33 patients of confirmed group, while the hemophagocytosis was found in 4 patients of excluded group. The sensitivity of hemophagocytosis for the diagnosis of HLH was 76.7%, and its specificity was 77.8%. In conclusion, hemophagocytosis is a helpful marker for the diagnosis of most but not all HLH patients, however, the lack of hemophagocytosis does not mean to exclude the diagnosis of HLH.