We report about a case of atypical Kawasaki disease presenting with persistent fever and pneumonia unresponsive to antibiotic therapy. No other signs or symptoms suggestive for Kawasaki disease were evident at illness onset or thereafter. Despite the puzzling clinical presentation, diagnosis has been made timely. Intravenous immunoglobulin was administered within the first two weeks of fever, along with aspirin, but the child recovered only after steroid therapy. Although a transient coronary dilation was evident at diagnosis, no long term cardiac sequelae were observed. Atypical Kawasaki disease usually affects infants and toddlers and, due to delayed diagnosis and treatment, is strongly associated with an increased risk of permanent heart damage. The lung consolidation as clinical presentation of atypical Kawasaki disease has been described in a very few cases. A high index of suspicion is required in children with refractory pneumonia, fever and persistently high inflammatory markers. In these cases, pediatricians should consider atypical Kawasaki disease as a possible alternative diagnosis, because early clinical suspicion and prompt treatment with intravenous immunoglobulin and aspirin dramatically abate the high risk of permanent coronary damage.