Abstract: Objective To observe the specificity of mean corpuscular hemoglobin concentration（MCHC） in children with hereditary spherocytosis（HS）. Methods MCHC was measured in 50 children with newly diagnosed HS（HS group）,50 children with autoimmune hemolytic anemia（AHA group）,50 children with iron deficiency anemia（IDA group）,50 children with megaloblastic anemia（MA group） and 50 healthy controls（control group） using a Sysmex SE-9000 automatic blood cells analyser. Results The MCHC was significantly higher in HS group than in AHA,IDA,MA and healthy control groups（P＜0.05）.The cut-off value of MCHC was 355g/L.The sensitivity and specificity of MCHC in diagnosis of HS were 87.76% and 98.00%,respectively.No difference was found in MCHC among different groups（P＞0.05）. Conclusion Increased specificity of MCHC is of a significant value for the diagnosis of HS in children.