Abstract: Objectives To explore the clinical features and pathogenesis of Kawasaki disease (KD) combined with sterile pyuria. Methods A total of 420 patients diagnosed of KD were recruited and divided into pyuria group (95 patients) and control group (325 patients) according to urine routine examination on admission. The clinical data between the two groups were compared. Results There was no difference in gender, age, and the incidence of atypical KD (P all > 0.05). The levels of C-reactive protein, D-dimer concentrations, fibrinogen degradation products, alanine aminotransferase, aspartate aminotransferase, and urine retinol binding protein were higher in pyuria group than those in control group (all P > 0.05). No difference was found in the duration of fever before admission between two groups (P > 0.05). However, pyuria group had longer duration of fever after treatment with immunoglobulin (P < 0.05). There was no difference in coronary artery lesions and non-response in the first dose of immunoglobulin treatment between two groups (all P > 0.05). Conclusion The morbidity of sterile pyuria in KD patients was 22.6%. KD patients with sterile pyuria have more intense inflammatory response, markedly high coagulation condition, and mild or subclinical renal damage.