Kawasaki disease is a systemic vasculitis that presents with stable vital signs. Although it is well known that Kawasaki disease can cause myocarditis, tachycardia and heart failure during the acute stage, Kawasaki disease shock syndrome (KDSS) has recently been described. It is characterized by hypotension, signs and symptoms of poor perfusion and a shock-like state. We report the case of a 7-year-old boy with KDSS who presented with persistent fever, conjunctival injection, laterocervical lymphadenitis, and echocardiography showing signs of myocardial dysfunction and shock. The patient's hemodynamic status markedly improved with immunoglobulin therapy. Early recognition of KDSS can be challenging; however, delay in diagnosis may increase the risk of coronary artery abnormalities and death.