Kawasaki disease (KD) is a medium vessel vasculitis with predilection to cause coronary artery abnormalities. KD is now the most common cause of acquired heart disease in developed countries. Thrombocytosis is consistently found in patients with KD, usually in 2nd to 3rd week of illness. Thrombocytopenia has occasionally been reported in the acute phase of KD. An increase or decrease in platelet number in patients with KD was initially considered to be a benign phenomenon. However, recent literature on platelet biology in KD has suggested that platelets are not only increasing but are rather activated. This phenomenon has been found to increase the risk of thrombosis in these patients. Similarly a fall in platelet counts during acute stage of KD has also been found to be associated with increased severity of disease. In this review, we update on the current best understanding about pathogenic role of platelets in patients with KD.