Objective　To explore the clinical features, diagnosis, and treatment of Kawasaki disease in neonates. Methods　The diagnosis and treatment of incomplete Kawasaki disease in one case were retrospectively analyzed. The related literatures were reviewed. Results　Male infant had persistent fever with rash at 26 days after birth. The anti-infective treatment was ineffective. No abnormality was found in the first coronary artery ultrasonography. However, coronary artery dilatation was confirmed by ultrasonography after skin peeling at fingertips. After the treatment of gamma globulin and aspirin, the symptoms of fever and rash were improved. The left dilated coronary artery returned to normal after 3 month. The size of coronary artery and the corresponding indexes were normal in 6-months, 1-year and 3 year follow-ups. Conclusion　Neonatal Kawasaki disease is rare and atypical.