Dermatomyositis, a syndrome closely related to the other connective tissue diseases has a wide clinical spectrum involving primarily muscle at the one end, but presenting a more generalized disorder with skin and visceral involvement at the other. Multiple etiologic agents probably exist, but the present series of 13 children implicated unknown agricultural factors. The diagnosis is essentially a clinical one, the laboratory findings being either nonspecific or unreliable. Renal biopsy revealed striking but nonspecific changes in all 6 patients studied, often without other evidence of renal disease. Current therapy does not contribute significantly to the prognosis, although steroids frequently yield dramatic results in the acute stage, and mechanical respiratory aids may be life-saving.