Generalized edema can occur during the course of juvenile dermatomyositis. In this article, a 4-year-old boy with generalized nonpitting edema and proximal weakness is reported. Characteristic cutaneous lesions, laboratory tests, results of electromyography, and magnetic resonance imaging findings resulted in a diagnosis of juvenile dermatomyositis. He was treated with corticosteroids and methotrexate. It is concluded that the generalized edema may appear as the presenting cutaneous manifestation of juvenile dermatomyositis.