A review of 96 patients with the established diagnosis of childhood dermatomyositis revealed that 15 patients required surgical therapy in addition to diagnostic biopsy. Four of the patients had perforation of the esophagointestinal tract, and 11 required surgical therapy for abscesses, calcific deposits, or treatment of pneumothorax. The life-threatening lesions were perforations of the esophagus and intestine. The best surgical therapy is closure, resection, and adequate drainage. Prognosis depends on treatment of the underlying medical disease.