Jo-1 syndrome is a disease recently described, included on the list of connective tissue diseases. Its clinical features are myositis and/or pulmonary fibrosis associated to the presence of precipitant antibodies against intracellular enzyme call histidine T-RNA synthetase. This antibody is related to pulmonary fibrosis associated to myositis and some scientist gave predictive value on the onset of pulmonary fibrosis in patients with myositis. However, isolated association of pulmonary fibrosis have been exceptionally described. A patient with severe interstitial pulmonary affliction and positive Jo-1 antibody without myositis is presented. The actual knowledge of the disease and its association is reviewed.