The DRESS (Drug Reaction with Eosinophilia and Systemic Symptoms) syndrome, characterized by fever, skin rash, lymphadenopathy and internal organ involvement is a delayed-type drug-hypersensitivity reaction that is life-threatening and rarely seen in childhood. A five-year-old girl was admitted with complaints of extensive rash and fever a month after the onset of treatment with carbamazepine for a diagnosis of epilepsy. Physical examination revealed fever, an extensive maculopapular rash, submandibular lymphadenopathy and hepatomegaly. The patient was diagnosed with the DRESS syndrome as a result of the clinical and laboratory findings (presence of atypical lymphocytes, eosinophilia and hypertransaminasemia) and treated supportively together with systemic corticosteroids and intravenous immunoglobulin (IVIG). The DRESS syndrome should be considered in the differential diagnosis in children with a history of drug use presenting with fever, skin rash, lymphadenopathy and internal organ involvement. The basic principles in the treatment include early diagnosis, termination of suspected drugs and supportive therapy. Systemic corticosteroids and IVIG therapy are recommended, especially in severe cases

Keywords: Anticonvulsants, Child, DRESS syndrome, Hypersensitivity, Carbamazepine