Kawasaki disease shock syndrome (KDSS) refers to Kawasaki disease (KD) patients who present more than 20% decrease in systolic blood pressure compared to healthy individuals of the same age, or to those patients who show peripheral blood circulation perfusion disorder. KDSS may have varied clinical courses and could be easily ignored and misdiagnosed. Yet, this syndrome could be deleterious, especially when combined with coronary artery injury or multiple organ dysfunctions. Therefore, early diagnosis of KDSS through specific clinical manifestations is critical. This manuscript summarizes the clinical features and treatment methods of 11 children with KDSS who were able to fully recover in our hospital. The results shed lights on understanding the early identification of KDSS and could assist in reducing the complications of KD and improving the quality of life in KDSS patients.