Kawasaki Disease (KD) is an acute systemic vasculitic disorder of childhood of unknown etiology. Initially KD was thought to be a benign disease, but later on it became obvious that cardiac manifestations are present in about 25-30% of patients and lead to death in about 0.5-2% of them. An early diagnosis and treatment are important to avoid an unfavourable prognosis. In recent years, a number of publications described patients in which the diagnosis was delayed because they did not fulfil the required criteria of KD. These forms of KD are known as atypical or incomplete KD. The aim of this work is to describe two cases of atypical KD observed at the Department of Pediatrics, University of Pisa during the year 1992.