To compare the treatment approaches and disease outcomes of children with JDM followed in two European tertiary care pediatric rheumatology centers.The medical notes of patients with JDM seen at Istituto Giannina Gaslini (IGG) of Genoa, Italy or Great Ormond Street Hospital (GOSH) of London, UK between January 2000 and December 2015 within 6 months after disease onset and followed for at least 6 months were reviewed. Demographic, clinical and therapeutic data were collected. At each visit, the caring physician was asked to rate subjectively the disease state.A total of 127 patients were included, 88 at GOSH and 39 at IGG. At 24 months, the median values of muscle strength and disease activity were at the normal end of the scale and around three quarter of patients were said to have inactive disease. Also at 2 years, 38.6% and 36% of British and Italian patients, respectively, had damage. Cyclophosphamide, azathioprine, infliximab, rituximab and mycophenolate mofetil were used more frequently by UK physicians, whereas cyclosporine, intravenous immunoglobulin and hydroxychloroquine were prescribed by Italian physicians.This study shows a significant difference in the choice of medications between pediatric rheumatologists practicing in the two centers. Despite this, a high proportion of patients had inactive disease at 2 years and there was a low frequency of damage: modern treatments have improved outcomes.