INTRODUCTION: The antisynthetase syndrome is characterized by the presence of myositis, interstitial lung disease, arthritis, Raynaud's phenomenon, mechanics hands and anti-Jo1 antibody (histidyl tRNA synthetase). The prognosis of this syndrome is closely related to the severity of lung disease. Myositis can occur several years after lung disease and some patients with interstitial lung disease associated with anti-Jo1 antibodies will not suffer from muscle disease. CASE-REPORT: We report the case of a 69-year-old man admitted to the medical intensive care unit for acute respiratory insufficiency related to rapidly progressive interstitial lung disease. Antisynthetase syndrome was diagnosed the presence of wrists' arthritis, 'mechanic's hands and anti-Jo1 antibodies. Despite the dramatic efficacy of corticosteroid therapy on ventilation parameters, the patient died from a Pseudomonas Aeruginosa nosocomial ventilator-acquired pneumonia. CONCLUSION: Our case emphasizes the importance to search for anti-Jo1 antibodies in the presence of interstitial lung disease. During the course of antisynthetase syndrome, the occurrence of interstitial lung disease is almost always constant and is correlated with poor prognosis.