Kawasaki disease (KD) is an acute inflammatory vasculitis that predominantly occurs in children under 5 years of age.1 It is associated with the development of coronary artery aneurysms (CAA) in 15–25% of untreated cases.2 Standard treatment consists of high-dose intravenous immunoglobulins (IVIG) along with aspirin.3 About 15% of patients do not respond to a single dose of IVIG and need retreatment.4 When ongoing signs of active disease are present, methylprednisolone pulses are often administered.5 If there is a lack of response, alternative anti-inflammatory medication such as infliximab or plasmapheresis have been suggested in individual case series.6 ,7 We report, for the first time, the beneficial use of an interleukin-1 receptor antagonist (IL-1RA) in relapsing KD.