Aim: To create awareness of Kawasaki disease in the dental community as it is a rare disease and some cases might go unnoticed due to lack of knowledge of the treating dentist. Thus proper knowledge helps in the timely diagnosis of the disease and decrease the mortality rate in these patients. Children who show the oral features of this disease should be treated seriously by the dentist. Background: Kawasaki disease is a rare acute systemic vasculitis disorder reported in the dental literature. Clinical features include high fever, redness of palms and soles of the feet, conjunctivitis, oropharyngeal mucositis and lymphadenopathy. The cardiac involvement in the form of myocarditis, aneurysms, pericarditis is also seen and is caused by inflammation of vessels of the heart. Case description: Here we present a rare case of an 8-yearold girl who presented to the department of Pediatric Dentistry with the chief complaint of recurrent painless swelling of the lower lip. This rare presentation of lower lip swelling has not been cited in the oral manifestation of Kawasaki disease before. Clinical significance: The disease has high mortality and morbidity rate if not treated early, and hence an early diagnosis and treatment are important in managing this condition. The oral findings are a characteristic feature of this serious disease, hence, many cases might first report to the dental clinician only. Dentists should always remain alert in handling patients having a history of Kawasaki disease because of the possibility of recurrence of the disease. As these patients have valvular heart defects, they might require prophylactic antibiotic treatment before the needed dental procedure. Conclusion: Despite this, there seems to be less aware of this disease among the dentist, hence this condition goes unnoticed leading to few citations of this disease in the dental literature.