Provisional hypomyopathic juvenile dermatomyositis is a subgroup of clinically amyopathic juvenile dermatomyositis provisional. The diagnostic criteria include: Classic dermatomyositis skin lesions – which have to be confirmed by biopsy –, no involvement of proximal muscles, subclinical involvement of these muscles and normal level of muscle enzymes. These criteria should be present for a minimum of 6 months and maximum of 24 months. Here a 13-year-old boy is presented who developed non-Hodgkin lymphoma 15 months after the diagnosis of his dermatomyositis.