Background Stevens-Johnson syndrome (SJS) is one of potentially fatal disorders that often occur after taking certain types of medication. There are reports of this disease after some infections. This paper presents the case of a young boy with idiopathic Stevens-Johnson syndrome. Case Report A six-year-old male patient with complaint of fever and skin lesions was transferred to emergency department of Bu-Ali Sina hospital (Center of Mazandaran province, Iran). After further evaluations (Physical examination and laboratory reports), the patient did not show any common causes of Stevens - Johnson syndrome. Fortunately he was discharged in a good condition after duration of treatment. Conclusion Further studies need to be done in the field of risk factors of Stevens-Johnson syndrome.