DRESS (drug rash with eosinophilia and systemic symptoms), also known as drug-induced hypersensitivity syndrome, is a severe, systemic drug reaction most commonly associated with aromatic anticonvulsants and sulfonamides. Patients typically present with fever, facial edema, cervical lymphadenopathy and a morbilliform eruption, which may progress to erythroderma. Hematologic abnormalities are a hallmark of the condition, including eosinophilia and atypical lymphocytosis. Visceral organ involvement typically manifests as hepatic dysfunction but may include lymphadenopathy, nephritis, interstitial pneumonitis, and myocarditis. Five to ten percent of patients with DRESS die from systemic complications, making timely recognition and treatment essential to prevent life-threatening manifestations. Myocarditis is a fatal and under-recognized manifestation of DRESS, which may occur long after the initial diagnosis. We review the literature of previously reported cases of DRESS and myocardial involvement, highlighting the presenting symptoms associated with cardiac involvement, treatments used, and the outcome for each patient. In addition, we offer an algorithm for early diagnosis, treatment, and subsequent monitoring of these patients.