Dermatomyositis is an inflammatory connective-tissue disease characterized by proximal muscle weakness and typical cutaneous lesions. Patients with dermatomyositis have an increased risk of malignancies. Here, we present the case of a 44-year-old male with progressive cutaneous rashes and proximal muscle weakness. He was diagnosed as having dermatomyositis after work-up. He received treatment with immunosuppressants, but the clinical response was limited. Further chest radiography demonstrated a nodular opacity at the right hilum. Bronchoscopic biopsy was performed and the pathology report revealed small cell lung cancer. After chemotherapy, the cutaneous lesions showed significant improvement. Since dermatomyositis is often a para-neoplastic syndrome, all patients with this disease should undergo complete work-up for malignancy.