A patient with sarcoidosis who presented with acute inflammatory myopathy is described. The patient had no symptoms other than those of the muscle involvement, with elevated serum levels of creatine kinase. Muscle biopsies revealed epithelioid granulomas, consistent with sarcoidosis. There was a dramatic response to a moderate dose of corticosteroids, but relapse occurred after the dose was tapered. We suggest that sarcoidosis be considered in the differential diagnosis of inflammatory myopathy, and that patients with acute sarcoid myositis be maintained with at least a moderate dose of corticosteroids for at least 6 months.