ABSTRACT Antisynthetase syndrome (ASS) is a rare, chronic autoimmune disease and is a sub-group of idiopathic inflammatory myopathies. The hallmark of this disorder is the presence of autoantibodies against the aminoacyl-transfer ribonucleic acid (tRNA) synthetase. The patient presents with fever, exanthema on the hands (also referred to as mechanic’s hands), myositis, and/or interstitial lung disease (ILD), and/or articular involvement. Nearly 70% of patients with ASS present with signs and symptoms of ILD. We are reporting a case of a patient who was having ILD, who turned out to have ASS on further workup. ASS as the causal diagnosis is missed many times. As an early and exact diagnosis has crucial therapeutic and prognostic implications, knowledge about this syndrome and a high level of suspicion is of utmost importance. Keyword: Antisynthetase syndrome (ASS), Autoimmune disease , interstitial lung disease