Kawasaki disease (KD) is one of the most common vasculitic disorders in children. The diagnosis is made based on the presence of clinical criteria. We present an exceptional case that fulfilled the clinical criteria of KD except the presence of fever. We consider that the criteria should be revised, with fever not to be considered as an obligate criterion. The similarities of KD with recurrent toxin-mediated perineal erythema are discussed, the latter being a possible attenuated form of KD.