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BACKGROUND: Orbital polymyositis associated with giant cell myocarditis rarely has been reported in the literature. The authors report the clinical, neuroradiographic, and histopathologic features of the only patient to survive this usually fatal syndrome after cardiac transplantation. FINDINGS: This 22-year-old white woman presented in 1991 with periorbital redness, swelling, and pain in both eyes that was unresponsive to antibiotic therapy. Results of her examination were significant for limited extraocular movements, ptosis, erythema, edema, chemosis, and exophthalmos. Electrocardiogram and chest x-ray were normal. Orbital computed tomographic scan showed swelling of the extraocular muscles up to and including their insertions. The patient was given the diagnosis of orbital polymyositis and her condition improved clinically and radiographically while taking parenteral steroids. One month after discharge, the patient was in cardiogenic shock. Endomyocardial biopsy showed giant cell myocarditis, and the patient underwent emergent cardiac transplantation. Despite a complicated postoperative course, the patient has done remarkably well. CONCLUSION: Although this disorder is rare, this case suggests the need for a high index of suspicion for giant cell myocarditis in patients with inflammatory orbital polymyositis. In non-Graves orbital polymyositis the patient should be questioned and instructed concerning the signs and symptoms of congestive heart failure. Chest x-ray, Holter monitoring, and electrocardiogram also should be performed and be repeated with an echocardiogram if there are any cardiac symptoms. In addition, early endomyocardial biopsy should be considered in the proper clinical setting, allowing timely diagnosis and expeditious cardiac transplantation