Stevens-Johnson syndrome (SJS) is a mucocutaneous disorder induced by an immune-complex-mediated hypersensitivity reaction. Nearly half of cases are caused by a reaction to drugs or appear during viral infections and malignancies. A very few cases are caused by a bacterial infection (Streptococcus) or Mycoplasma pneumoniae. Graft versus host disease is another well-established cause, independent of drugs. No specific etiology has been identified in up to half of cases. We report a 54-year-old man with SJS induced by carbamazepine. Reported patient had prodromal symptoms like fever, headache and polyarthralgia, which preceded mucocutaneous lesions by 3 days. Physical examination on admission, revealed asthenic male with a temperature of 37.2 degrees C and generalized dermatitis with positive Nikolsky sign, large erosions of the palms and soles, onychomadesis, numerous oral and vermilion border of the lips erosions. The patient was administered systemic steroidotherapy and carbamazepine dose was gradually decreased and finally replaced with valproic acid and valproate sodium. During the hospitalization, temperature normalized and the skin lesions disappeared after 3 weeks of treatment.