The association between interstitial lung disease and polymyositis/dermatomyositis is well known. It severely affects patients' quality of life, worsens prognosis and represents a major risk factor for premature death. Current treatment is unclear and therapeutic options are based on case series. We report the case of a 15-year old female diagnosed with end-stage lung disease associated to polymyositis who received a double lung transplant after 20 days of extracorporeal membrane oxygenation. She died 9 months later and microscopic post-mortem findings revealed recurrence of interstitial lung disease. This is the first time that recurrence of polymyositis-associated lung disease following lung transplantation is described in the literature.