A 4-month-old infant residing in a foreign country, presented with high fever associated with poor general condition, bilateral conjunctivitis and livedo reticularis. Tests showed elevated inflammation markers with negative blood cultures, chest X-ray, abdominal ultrasound and lumbar puncture. After the failure of intravenous antibiotic therapy, systemic inflammatory disease was suspected. Therefore, the therapy with methylprednisolone (mPDN) and acetylsalicylic acid (ASA) was initiated and led to rapid defervescence. Due to a relapse of fever upon discontinuation of steroid therapy, and after two negative echocardiograms, intravenous immunoglobulin infusion was performed after 13 days of fever. The family then decided to discharge themselves from the hospital and take their child to our hospital where several investigations were carried out, revealing an increase in inflammation markers with no apparent focus. Given the strongly suspicious presentation for incomplete Kawasaki syndrome, weekly echocardiographic monitoring was started, which, about a month after the onset of symptoms, showed coronary aneurysms, initially small size, then medium size. As a result, therapy with anakinra was initiated with excellent clinical and radiological response.