Kawasaki disease (KD) is a common acute vasculitis in children, while Kawasaki disease shock syndrome (KDSS) is a rare and serious manifestation of KD. In recent years, KDSS has gradually attracted the attention of clinicians, because it can appear in the early stage of the disease, with rapid progression and multi-system organ involvement. Early diagnosis of KDSS is difficult and is very easy to miss diagnosis and misdiagnose. This article reviews the research progress of KDSS in the diagnosis, early recognition, pathogenesis, treatment and prognosis in recent years.