Anti-synthetase syndrome (ASS) is an autoimmune disease characterized by the presence of auto antibodies against one of many aminoacyl transfer RNA synthetases and spectrum of clinical manifestations. Here we report a case of a 52 year old lady who had been extensively evaluated for progressive muscle weakness in a tertiary care center, diagnosed as Antisynthetase syndrome which was evidenced by the presence of antisynthetase antibody, dermatomyositis, Raynaud's phenomenon, arthritis and mechanics hand. Despite aggressive management with intravenous immunoglobulins, steroids and immune suppressors she died because of underlying respiratory failure causing difficulty in weaning off ventilator.