We report the clinical course of three patients with refractory juvenile dermatomyositis (JDM) who were treated with tacrolimus. All three children had extensive skin disease and severe muscle weakness and were corticosteroid dependent. All three patients showed impressive improvement of mainly the cutaneous lesions. Furthermore, overall disease activity decreased, all children became more physically active, and corticosteroid treatment could be tapered. However, none of the patients showed recovery of muscle strength, which was most likely due to irreversible muscle damage related to the long-standing myositis and/or high-dose steroid treatment. Patients were followed up for 7 to 9 months after the introduction of tacrolimus. No adverse effects were seen. These cases demonstrate that tacrolimus has beneficial effects in children with refractory JDM, especially in those with severe cutaneous manifestations.