A case-control study was performed to ascertain clinical features of children who had been diagnosed as Kawasaki disease shock syndrome (KDSS), a severe condition related to Kawasaki disease (KD). Hospitalized patients were selected in Nanjing Children’s Hospital. Demographic characteristics, clinical presentation, laboratory data, cardiovascular findings, and therapies were analyzed. Compared with the control group, KDSS patients were older and had more serious skin rash. The proportions of leukocytosis, neutrophilia, and hypoalbuminemia was higher, as was the level of while blood cell count, C-reactive protein, brain natriuretic peptide, and ferroprotein. KDSS patients had higher incidence of arrhythmias and more severe coronary artery involvement. All case patients received aspirin, glucocorticoid, and intravenous immunoglobulin, 33.3% required albumin, and 90.4% needed vasoactive infusions. In conclusion, KDSS patients may have more serious inflammatory responses in the acute phase. Short-term use of glucocorticoid may be important in inhibiting the inflammatory response. Albumin and vasoactive drugs are useful to rescue shock.