Juvenile Dermatomyositis is a rare idiopathic autoimmune and inflammatory myopathy and vasculopathy whose hallmarks are symmetrical proximal muscle weaknesses and a characteristic rash. Only few cases have been reported in West Africa subregion. We present a 14-year old Nigerian girl with clinical and histopathologic features of definitive juvenile dermatomyositis based on EULAR/ACR classification criteria but probable Juvenile dermatomyositis according to Bohan and Peter criteria. The patient had normal aspartate and alanine aminotransferase levels. Creatine kinase, Lactate dehydrogenase and aldolase which are not available in our center could not be evaluated. There was remarkable clinical improvement 3 weeks after the onset of systemic corticosteroid therapy. Our case highlights that relying on these normal enzyme values, especially where muscle biopsy and EMG are not available as is the case in most centers in developing countries, would have resulted in missed diagnosis using Bohan and Peter criteria.