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Juvenile dermatomyositis (JDM) is a systemic, autoimmune inflammatory muscle disorder and vasculopathy of unknown origin that affects children younger than 18 years. JDM primarily affects the skin and the proximal skeletal muscles. Globally, JDM is rare; in Nigeria only one case has been reported. In general, children with JDM are able to lead normal lives with full recovery when diagnosis and initiation of appropriate therapy is early. A 12 year old girl presented with proximal myopathy, skin rash, elevated muscle enzymes and typical muscle histology. The diagnosis of JDM was delayed despite her having accessed several health facilities. Response to steroids was incomplete as she already had disabilities (muscle wasting with inability to walk) before treatment was commenced. We highlight the challenges of management in a low resource setting which included delay in diagnosis, lack of diagnostic tools, poor referral system and lack of health funding. The need for awareness and a high index of suspicion among health care workers is emphasized to ensure early referrals for early diagnosis/early initiation of appropriate therapy and improved outcome.