Kawasaki disease (KD) is an acute systemic vasculitis that occurs primarily in children and rarely in adults, possibly after bacterial or viral infections in genetically susceptible hosts. KD may frequently be undiagnosed especially in adult patients without the presence of all the classical clinical criteria (incomplete or atypical KD). In addition, many differential diagnoses could be considered. Here, we report a case of KD in an adult patient with clinical features characteristic of the classical form. KD requires a long-term management in both paediatric and adult patients, in order to avoid complications that could follow both the acute and retrospective diagnoses of KD.