Clinically amyopathic dermatomyositis (CADM) is a rare condition characterized by dermatomyositis skin lesions without clinically apparent muscle involvement. Respiratory involvement is common, occurring in about half of the cases. Spontaneous pneumomediastinum (PnM) is a rare, and often fatal, complication of CADM. We report a case of a 61-year-old female patient who was diagnosed with anti-melanoma differentiation- associated gene 5 antibody-associated CADM and interstitial lung disease. She developed an extensive spontaneous PnM with subcutaneous emphysema. The patient was treated with a conservative approach which was, initially, successful in reducing the size of the PnM. However, the patient died from an eventual nosocomial pneumonia requiring mechanical ventilation. This case illustrates that improving the management of CADM associated PnM, remains a major unmet need.