Stevens-Johnson syndrome (SJS) and toxic epidermal necrolysis (TEN) are rare life-threatening conditions almost exclusively attributed to drugs. The incidence in children is lower than in adults and has a better outcome. Mycosplama pneumoniae infection may be involved in some cases of paediatric SJS. The main etiologic factors for both SSJ and TEN are sulphonamides and anticonvulsants, followed by penicillins and non-steroidal anti-inflammatory drugs. In rare instances, paracetamol is the only suspected drug. By contrast with adults, allopurinol, oxicams and nevirapine are not identi fied as causative agents in children, probably due to differences in drug prescriptions. The only aspects of treatment that have been proved to improve survival are the rapid withdrawal of the suspected offending drugs and an optimal supportive therapy with emphasis in nutritional support, accompanied by management of denuded skin areas. The use of specific therapies remains controversial.