A wide range of conditions can mimic polymyositis. Thus, diagnosing this condition can be a challenge. Although no single criterion is diagnostic of polymyositis, the following criteria have been proposed and widely used: (1) symmetric proximal muscle weakness; (2) characteristic violaceous rash on the hands, elbows, and knees; (3) increased muscle enzymes in the serum; (4) characteristic electromyographic findings (insertional activity, fibrillation potentials, motor unit potentials of increased frequency and decreased duration, and normal conduction velocity in nerves); and (5) muscle biopsy specimen with characteristic inflammatory and myopathic changes. Although polymyositis primarily involves muscle, up to 20% of patients may have extramuscular problems. The main treatment for polymyositis is high-dose corticosteroids. In corticosteroid-resistant patients, methotrexate is often effective. In this report, case histories are presented to highlight the usefulness and the limitations of the common diagnostic criteria for polymyositis. © 1990, Mayo Foundation for Medical Education and Research. All rights reserved.