Background and design: Drug hypersensitivity syndrome is characterized by fever, lymphadenopathy, skin rash and internal organ involvement. Various drugs may cause this type of adverse drug reaction but it is mainly associated with the aromatic antiepileptic drugs carbamazepine, phenytoin, phenobarbital and primidone. The incidence is approximately 1 in 3000 exposures, and it usually starts between 1 week and 3 months after initiation of therapy. It is a potentially fatal reaction mainly as a result of internal manifestations, such as hepatic, renal or bone marrow involvement. Skin rash can range from maculopapular I morbilliform eruption to exfoliative dermatitis or toxic epidermal necrolysis. The syndrome can mimic infectious, neoplastic or connective tissue disorders. We herein present a case of carbamazepine-induced drug hypersensitivity syndrome who developed fever, lymphadenopathy, exfoliative dermatitis, and hepatitis 50 days after initiation of therapy. The reaction worsened even after cessation of carbamazepine therapy therefore methylprednisolone 40 mg/day i.m. was started, and an improvement could be achieved within one month. In conclusion, this rare but severe drug reaction should always be considered in the differential diagnosis of any skin eruption that would occur in patients receiving antiepileptic drugs.