Amyopathic dermatomyositis (ADM) causes a severe pulmonary disease in 50% of patients and no validated therapeutic option is available. Mycophenolate mofetil permitted to control interstitial lung disease and pneumomediastinum in a patient with ADM. This drug has recently been used to treat interstitial lung disease in ADM, but its effectiveness has not been previously reported in case of pneumomediastinum. Patients with ADM need an accurate pulmonary surveillance and in case of pulmonary complications mycophenolate mofetil could be a new therapeutic option.