Severe skin reactions complicating tumor necrosis factor-alpha (TNF-α) antagonist therapy were somewhat reported in adults. We report a case of 24-month-old boy with refractory Kawasaki disease who developed toxic epidermal necrolysis (TEN) after one dose of 5mg/kg infliximab. The patient responded to intravenous hydrocortisone and was able to discharge without any other serious complications. The clinicians should be aware of the potential for severe skin reactions from the use of TNF-α antagonists in children.