Introduction: Presentation of Dermatomyositis with Interstitial Lung Disease (ILD) is not uncommon. Case descrip-tion: A 50 years old lady with dyspnoea of 1 month. On examination patient had heliotrope rash around face with gottron papules and fine crepitations bibasally over lung auscultation with proximal weakness of the limbs. High Resolution Computer Tomography of Chest(HRCT of Chest) revealed ground glass appearance with fibrotic changes in both lungs. Blood test serum ANA 1:160, Anti MDA5 strong positive, Anti Ro 52 positive with Serum dsDNA nega-tive. Skin Biopsy-compatible with cutaneous manifestation of dermatomyositis. EMG revealed there is electrophysio-logical cause of myopathy. Lung function test was unable to perform due to severe pain. Investigate for the possibility of underlying malignancy were taken with Serum Tumour Markers, Nasoscopy, ultrasound of pelvis, OGDS and colonoscopy. Did not reveal lesions suspicious of malignancy. Patient treated by using tablet prednisolone 50mg once daily with tapering done and tablet azathioprine 100mg once daily. Planned for IV Methylprednisolone if wors-ening of disease. Patient passed away due to rapidly worsening ILD. Conclusion: Amyotrophic Dermatomyositis with interstitial lung disease(ILD) diagnosis can be confidently diagnosed with clinical correlation and Serum AntiMDA5.