Early diagnosis of Kawasaki disease (KD) is critical to allow prompt initiation of treatment and avoid cardiac complications. All children with KD have fever accompanied by clinical signs, with four of the five classic criteria for complete KD being mucocutaneous, thus creating an important role for dermatologists. Moreover, dermatologists must be familiar with other dermatological findings that are not included in the American Heart Association classification criteria but can support the diagnosis, particularly in incomplete forms of the disease. We review the skin manifestations described for KD and perform an overview of pathophysiological advances and new treatments.