<p>Kawasaki disease (KD) is the most common systemic vasculitis in childhood and represents a&#xa0;significant pediatric challenge, particularly due to potential coronary artery complications. The American Heart Association has updated the guidelines on Kawasaki disease 7 years after the last version. The diagnosis is still based on clinical findings and a specific biomarker is still lacking. The required duration of fever was reduced from 5 to 4 days, which in experienced hands can even be after 3 days. The main symptoms are unchanged but do not have to be simultaneously present. The Z scores are recommended for echocardiography but without a uniform assessment model. The standard treatment still includes i.v. immunoglobulins (IVIG) and acetylsalicylic acid (ASS) in a mid-range dose (30–50mg/kg body weight). High-risk patients (e.g., young age, Asiatic ethnic background, increased inflammation values, dilated coronary vessels) can benefit from an intensified treatment with corticosteroids or infliximab, whereby the American guidelines do not provide any clear risk stratification. In IVIG-resistant cases a second administration of IVIG and/or corticosteroids is recommended. Infliximab can reduce the treatment resistance but does not show a safe protection from coronary aneurysms. Overall, it is emphasized that an early diagnosis and treatment are decisive to avoid complications and an interdisciplinary treatment is recommended. The article also addresses controversial aspects of treatment escalation and provides an outlook on unresolved questions and future developments in the management of this complex disease.</p>