Idiopathic pulmonary fibrosis and pulmonary fibrosis associated with systemic diseases are the most frequent diffuse interstitial pulmonary diseases. They slowly but irrevocably progress towards terminal respiratory failure. They can also be complicated by severe acute respiratory failure and admission in the intensive care unit can be discussed. Despite invasive mechanical ventilation, anti-infectious and immunosuppressive treatments, the disease carries a high mortality rate. We report and discuss the case of a patient with idiopathic, pulmonary fibrosis (UIP) who underwent rapid clinical deterioration.