Objective To explore the clinical features and treatment of Kawasaki disease shock syndrome (KDSS) with acute kidney injury (AKI). Method The clinical data of KDSS with AKI in a child was reviewed and analyzed. Results A 12 -year-old girl presented with fever and vomiting at the onset of Kawasaki disease. Acute kidney injury developed gradually after septic shock was diagnosed. The fever subsided 10 days later, urine output recovered after 5 continuous renal replacement treatment (CRRT), and blood pressure stabilized. The urea nitrogen and creatinine returned to normal. Later re-examination of cardiac color Doppler echocardiography indicated coronary artery dilation, and then the diagnosis was revised as KDSS. The patient was treated with aspirin and discharged from the hospital. Long-term follow-up showed that the coronary dilatation disappeared. Conclusions KDSS should be diagnosed from septic shock at the early stage. Once AKI is complicated, the active use of CRRT can effectively improve the prognosis.