Introduction. High flow nasal cannula oxygen therapy (HFNC) has become frequent in the treatment of patients with acute hypoxemic respiratory failure. Methods. Eleven patients with acute exacerbation of fibrotic interstitial lung disease (ILD) were treated with HFNC after failure of conventional therapy (SatO2< 90% offering 100% FiO2 by non-rebreathing mask or noninvasive ventilation). Results. Ten patients had success with HFNC (not requiring orotracheal intubation) during emergency department admission. HFNC significantly improves clinical variables after 2h: respiratory rate decreased from 33 ± 6 breaths/min to 23 ± 3 breaths/min; PaO2 increased from 48.7 (38 - 59)mmHg to 81.1 (76 - 90) mmHg; PaO2/FiO2 ratio increased from 102.4 ± 32.2 to 136.6 ± 29.4; SatO2 increased from 85 (66 - 92)% to 96 ± (95 - 97)%. HFNC could be an effective alternative in the treatment of acute respiratory failure from acute exacerbations of fibrotic ILD.