Drug-induced hypersensitivity syndrome (DIHS) is a rare and potentially fatal reaction characterized by fever, rash, and internal organ involvement that typically occurs between 3 and 6 weeks after commencing the drug. We describe such a case in a 26-year-old woman, who developed fever, exfoliative erythroderma, facial edema, cervical lymphadenopathy, hepatitis, and leukocytosis 6 weeks after commencing carbamazepine for lower back pain. Her serum angiotensin-converting enzyme level was also raised to 144 U/L (8-52 U/L). Skin biopsies demonstrated an unusual superficial dermal perivascular inflammatory infiltrate, which included conspicuous granulomas mixed with moderate numbers of lymphocytes. Eosinophils were not a feature. Her carbamazepine was withdrawn, and oral prednisone was commenced initially at a dose of 1 mg.kg.d and slowly weaned over a 6-week period. Her fever, rash, facial edema, and hepatitis gradually resolved within this period, and her serum angiotensin-converting enzyme level returned to within the normal range. Although the patient's clinical course was consistent with a DIHS, it was accompanied by a previously unreported finding of a superficial granulomatous dermatitis. Granuloma formation as a sequel to medication use is a feature of interstitial granulomatous drug reaction. However, interstitial granulomatous drug reaction consists of localized violaceous plaques with a predilection for skin fold areas and liver function abnormalities have not been described. Granulomatous inflammation in other organ systems, including the liver and kidney, has also been described after the use of carbamazepine, but these reactions are not associated with the systemic manifestations observed in DIHS.