Approximately one-third of adult patients with dermatomyositis develop malignancy, usually within 1 year of diagnosis of dermatomyositis. This phenomenon is not seen in patients with juvenile dermatomyositis. We are reporting a case of B-cell lymphoma arising from a calcinotic lesion in a 14-year-old girl who had been diagnosed with juvenile dermatomyositis 3 years prior. Our review of the literature revealed only one other case of malignancy arising from an area of calcinosis.