In patients with juvenile dermatomyositis/polymyositis (JDM/PM), malignancy is a rare phenomenon. An extensive workup for neoplastic disease is not routinely indicated, but should be considered if unusual physical findings are noted at JDM/PM diagnosis or during follow-up period. The objective of this literature review was to assess for any association between, and disease profile of, JDM/PM and lymphoma in childhood. Risk determinants of the possible development of lymphoma in the pediatric population with JDM/PM appear to be the degree and duration of inflammatory activity with chronic B-cell activation and/or antigen stimulation; a paraneoplastic relationship is unlikely.