Introduction: Stevens-Johnson syndrome(SJS)and toxic epidermal necrolysis(TEN)in children are rare,but potentially life-threatening mucocutaneous reactions.The exact pathogenesis of SJS/TEN in children is not well established,includes infection,medical genomics and immunological mechanisms.Case report: We report here a rare case of SJS/TEN triggered by a combination of infection and drug in a 6-year-old boy.The child developed SJS/TEN with a progressive multiple skin lesions noted all over the body with liver and gastrointestine involvement one week after the initiation of upper respiratory infection therapy.All related drugs,such as penicillins and saridon,were discontinued,and intravenous methylprednisolone,intravenous immunoglobulin,fluid supplement,special wound care,and supportive medical care for SJS/TEN were administered.He was discharged in a stable condition on the 24th day.Conclusion: Our case suggests that prodromal infection history,together with a drug-drug interaction between amoxicillin and saridon may contributed to the cause of SJS/TEN in children.Purpose of Review: This study summarizes up-to-date insights and understanding of the pathogenesis of SJS/TEN and provides an update on the treatment of these conditions in children.We hope that this report will add to the existing meager body of literature and throw some light on the occurrence and manifestation of this condition.