We report the case of an 11-year-old boy with proximal myopathy, heliotrope, and Gottron papule-like rashes. Serum chemistry revealed muscle enzyme elevations, whereas muscle biopsy histology showed necrosis and inflammation, which were in keeping with juvenile dermatomyositis. Plain radiographic examination of the thigh 3 weeks after commencing treatment with prednisolone was normal. The aim of this presentation is to highlight the diagnostic challenges posed by this rare condition in a resource-limited setting and to underscore the need for prompt diagnosis and appropriate management. We hope that this report will assist physicians practicing in similar settings to make a prompt and accurate diagnosis when confronted with the same disease.