Kawasaki disease is the most frequent reason for acquired heart diseases in developed countries. The disease may develop with many clinical pictures and the most important disease-related complication is coronary arterial aneurysm. The sooner the diagnosis is made and treatment is initiated, the lower the risk for its complications to arise. Atypical Kawasaki disease is characterized by organ and system involvement, which do not clinically meet the disease criteria, are unexpected or not frequently seen under normal circumstances. Approximately 7-10% of the patients show atypical involvement. In atypical patients, coronary arterial involvement is more frequent as a result of late diagnosis. This article presents the clinical conditions that may be seen as a result of atypical Kawasaki disease in the light of literature data.