Carbamazepine is an effective anticonvulsant and is considered the drug of first choice for the treatment of partial and secondarily generalized seizures. Although carbamazepine is well tolerated, many side effects have been reported in the literature. The majority of these adverse effects are transient and do not lead to the discontinuation of the therapy.We present a case of a female child, aged 11 years and 6 months, who showed an anticonvulsant hypersensitivity syndrome induced by carbamazepine. This syndrome is a rare, potentially life-threatening adverse drug reaction. The patient developed a cutaneous nonpruritic rash, associated with high fever, diffuse lymphadenopathy, and arthralgias on the knees and the ankles with local signs of arthritis. Laboratory examination showed a lymphocytosis, mild thrombocytopenia, marked eosinophilia, and high transaminases.Corticosteroid therapy (betametasone 0,5 mg x 3 day) was started and carbamazepine was gradually withdrawn changing to valproic acid, with complete control of the seizures. The fever and the rash reduced gradually, beginning from the face and then disappearing completely after 10 days. Laboratory results showed a clear improvement: after 7 days the patient showed a complete normalization of the above parameters, except for transaminases. The complete normalization of these enzymes was observed after 2 weeks from the disappearance of the skin rash.Carbamazepine (CBZ) is an effective anticonvulsant that has shown clinical efficacy in partial and secondary generalized seizures; it is an iminostilbine with a tricyclic structure (l, 2).CBZ is effective in affective disorders and trigeminal neuralgia, but its principal use is in the therapy of epilepsy; this drug is used for the treatment of partial elementary, partial complex, secondarily generalized and tonic-clonic seizures (3). A major advantage of carbamazepine is in its low potential for producing adverse behavioral and cognitive side effects (4).Although CBZ is well tolerated and global patient evaluation shows good results, many side effects have been reported in the literature. The majority of the adverse effects of CBZ includes nausea, drowsiness, vertigo, ataxia, blurred vision, diplopia. Very few patients require discontinuation of CBZ because of these side effects; Adverse reactions are most frequently transient and do not lead to discontinuation of therapy.Idiosyncratic reactions, including skin problems, have been frequently described and vary from 25% to 50% (5-9).Among the dermatologic manifestations, rashes are the most frequent finding. Their severity is generally mild and the discontinuation of the treatment solves these cutaneous features. Among the severe skin reactions (l0) anticonvulsant hypersensitivity syndrome (ARS) is a very rare