We report on an unusual case of a 40-year-old Caucasian male displaying severe Kawasaki-like symptoms. The disease lasted for seven years before diffuse coronary aneurysms occurred, leading to the patient’s death, despite ongoing treatment by intravenous immunoglobulins (IVIGs). The patient had also been suffering from a disabling inflammation of the spine, which was reported to have started at the onset of the disorder. Whereas neither NSAIDS, nor high doses corticosteroids, or anti-TNF drugs had a clear effect, the clinical features of spinal inflammation were highly sensitive to IVIGs, and were attributed definitively to HLA-B27-negative axial spondylarthropathy after bone scan and magnetic resonance imaging disclosed typical enthesitis of both heels and bilateral sacroiliitis.