RATIONALE: Lung transplantation in rapidly progressive interstitial lung disease associated with antimelanoma differentiation-associated gene 5 antibody positive may be a rescue therapy in case of medical treatment failure and impossible weaning from the ventilatory support. PATIENT CONCERNS: A 59-year-old man with cough, expectoration, and fever for more than half a month, which aggravated with dyspnea for 4 days. DIAGNOSES: Rapidly progressive interstitial lung disease was confirmed through chest high-resolution computed tomography and antimelanoma differentiation-associated gene 5 antibody positive. INTERVENTIONS: The patient was given extracorporeal membrane oxygenation and double lung transplantation. OUTCOMES: The patient is currently undergoing rehabilitation in a general ward and follow-up. LESSONS: Lung transplantation should be considered when impossible weaning from the ventilatory support.