Introduction: Kawasaki disease is an acute, systemic vacuity that predominantly manifests in infants and young children. Severe systemic inflammation and vacuities can cause cardiovascular involvement, particularly coronary artery injury that includes dilatation or aneurysm. The final goal of treatment for acute Kawasaki disease، therefore، is to diminish systemic inflammation and vacuities as soon as possible, thereby, preventing progression to coronary artery injury and thrombosis.

Case Report: In this report we present a case of Kawasaki disease in a 7-year-old boy with prolonged fever and cervical adenopathy presented with erythema marginatum as a first skin presentation. We treated the patient with intravenous gamma-globulin (IVIG) by six day after admission. He developed cardiac and pulmonary involvement two days later.

Conclusion: Any skin rash is described in Kawasaki disease except for bullous and vesicular lesions. The common skin manifestation of Kawasaki disease are erythematous and maculopapular rash, although it may appear urticarial, scarlatiniform, erythema multiforme-like, or as erythroderma. Erythema marginatum had a limited differential diagnosis in pediatric patients. The major cause of erythema marginatum is rheumatic fever but there is no report of erythema marginatum in face and cervical region and Kawasaki disease should be considered in differential diagnosis of erythema marginatum in face and neck.