Adverse drug reactions are the major cause of morbidity and mortality worldwide. Cutaneous drug reaction is the most common type of adverse reaction. Toxic epidermal necrolysis (TEN) is a rare, life-threatening mucocutaneous disease, usually attributable to drugs. There is no proven therapy for TEN. The mainstay of therapy is immediate withdrawal of the culprit drug, using disease-modifying agents, and meticulous supportive care. Several disease-modifying agents have been used such as steroid, i.v. human immunoglobulin (IVIg), plasmapheresis. A 10-year-old epileptic girl was admitted with lamotrigine-induced TEN. She was unresponsive to steroid. Her condition deteriorated despite IVIg treatment. She was treated with N-acetylcysteine (NAC). To our knowledge this is the first report of a child with TEN, a potentially lethal disorder, treated with NAC. NAC may be effective for children with TEN.