Kawasaki disease is a systemic vasculitic inflammatory disease that is characterized by fever, rash, conjunctival congestion and non-purulent enlargement of the cervical lymph nodes. If left untreated, about 20% of children will develop coronary artery damage, which is the most common cause of acquired heart disease. The etiology of the disease is unclear and is presumed to be related to infections, including rickettsiae, staphylococci, streptococci, and retroviruses. For the pathogenesis, factors such as hyperimmunity, superantigens or heat shock proteins are considered to be involved. Inflammatory cells invade vascular tissue leading to vascular injury, especially in the coronary arteries, which can lead to the formation of aneurysms. Kawasaki disease is more common in infants and children, with a common age of onset of 6 months to 5 years, more common in boys, and a male to female ratio of 1.5:1. Most Kawasaki disease has a good prognosis with regular treatment. Treatment includes immunoglobulin, aspirin, anti-inflammatory and anti-platelet aggregation and anticoagulation therapy. Early treatment and regular follow-up of children diagnosed with Kawasaki disease can significantly reduce coronary artery aneurysms and mortality.