Abstract: Kawasaki disease （KD） is a febrile vasculitis disease, which mainly occurs in children all over the world. The etiology and pathogenesis of KD remain unknown, and its complications are diverse. Intravenous immune globulin （IVIG）-resistant KD is defined as persistent fever in patients after the initial treatment of IVIG and aspirin. In this article, two cases who were diagnosed with IVIG-resistant KD complicated with severe arthritis were reported. Magnetic resonance imaging （MRI） demonstrated joint effusion. After the second dose of IVIG was delivered, fever and joint pain symptoms were still not relieved. Eventually, low-dose oral prednisone was given, and then the fever was mitigated and the joint symptoms were relieved. The pathogenesis of KD-related severe arthritis remains elusive, which should be emphasized. At present, there is no treatment guideline for IVIG-resistant KD complicated with severe arthritis. Low-dose prednisone might become an optimal treatment of IVIG-resistant KD complicated with severe arthritis.