Introduction. Dermatomyositis is an idiopathic inflammatory myopathy with characteristic skin changes and a progressive muscle weakness. In the older age, the incidence of malignancy is higher in dermatomyositis. Paraneoplastic syndromes are described in 10-25% of adult patients with dermatomyositis. Case presentation. A 73-yearold man presented to our hospital with a history of 6 months skin lesions spread firstly on the scalp and later on the face, chest, shoulders, back and the upper limbs. He was also complaining of progressive muscle weakness of the arms and thighs during the last few weeks. There was no history of other constitutional symptoms such as weight loss, dyspnea or respiratory symptoms. On the dermatologic examination, we revealed heliotrope rash, a scaly scalp with inflammation, the specific shawl sign associated with photosensitivity over the sun-exposed sites, red nail folds, purple papules over the tops of the interphalangeal joints (Gottron papules). A chest x-ray and CT scan were performed with a result of a massive posteriorbasal expansive process on the left pulmonary field, hilar lymphadenopathy. The patient was diagnosed with paraneoplastic dermatomyositis based on the clinical and imagistic examination. He was urgently referred to the oncologist for establishing the malignant tumor diagnosis and to follow a specific therapy. Conclusions. Dermatomyositis is a rare disease and the physical findings may vary widely from case to case as patients may present differently. The association between dermatomyositis and malignancy is well documented. In this case we revealed classical dermatomyositis signs associated with lung malignant tumor.