Kawasaki disease (KD) is a systemic vasculitis of predominantly medium-sized arteries with frequent lesions of coronary arteries (CA), characterized by fever, cyclic self-stopping course, changes in the mucous membranes, skin and lymph nodes. Heart lesion determines the prognosis and outcomes of KD, both in the acute period and in the long term perspective. Most lethal outcomes of the acute and subacute period in KD develop suddenly and are a consequence of myocarditis, cardiac arrhythmias, thrombosis of aneurysmically altered CA, complicated by acute coronary insufficiency, development of myocardial infarction, acute heart failure. Objective of the research is he analysis of deaths in KD according to data of Morozov Children's City Clinical Hospital. 205 patients with KD aged from 1 month to 10 years 2 months (Me 19 months [9; 38,5]) in the 2014– 2019 period were examined. During this period, 5 deaths were recorded (4 boys, one girl), which amounted to 2,4%. In deceased patients as factors of an unfavorable KD outcome with CA lesion were: late initiation of pathogenetic therapy (4 children), impaired intravenous immunoglobulin administration regimen (1), male gender (4), infancy (3), formation of giant CA aneurysm (3), hypoalbuminemia (2), a significant increase in C-reactive protein by 7–22 times compared with normative indicators. The cause of death in all children was acute coronary insufficiency as a result of acute occlusive intracoronary thrombosis. © 2020, Pediatria Ltd.. All rights reserved.