Abstract: Objective: To find possible ways to reduce or avoid early misdiagnosis of Kawasaki disease.Methods: Forty firstly misdiagnosed cases of Kawasaki disease were retrospectively studied about the different diagnosis made in outpatient department and after hospitalization in combination with clinical manifestations and laboratory tests.Results: Most of the children had fever and respiratory symptoms;physical examination found rashes,conjunctive congestion,lip red with rhagadia,desquamating of finger and dactylus tips rather than hard dropsy,swollen cervical lymph nodes and decrustation of crissum's skin;laboratory tests showed high white blood cell counts and blood plaque counts;at the same time,erythrocytes sedimentation rates and C-reactive proteins levels were elevated;low albumin in blood,low plasma osmotic pressure and low sodium ion in blood were usually common.All the above clinical and laboratory findings were easily misdiagnosed as respiratory infection,septicemia,drug rashes,cervical lymphnoditis,diarrhea,urinary tract infection or infectious mononucleosis;however,it was rarely misdiagnosed as measles,syndrome of streptococcus infection,conjunctivitis and juvenile rheumatoid arthritis or as convulsion with high fever.Proportion of untypical cases of Kawasaki disease was 32.5%,33.33% coronary vascular was damaged.Conclusions: It is important for medical professionals to understand the diseases so as to avoid early misdiagnosis.