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The authors present a clinical case of a 69-year-old male that was admitted in the emergency department with decreased strength in proximal muscle that started in the previous month. He also presented erythematous and pruriginous cutaneous lesions disseminated along the torso, with V-shaped, heliotropic rash, and rash in dorsum, face and upper limbs. Dermatomyositis was diagnosed. He was antinuclear antibodies positive and the analytical study showed elevated creatine kinase, lactate dehydrogenase and aldolase. The skin biopsy also confirmed the diagnosis. In the abdominal examination a mass was noticed in the right upper quadrant. Considering the possibility of the presence of a paraneoplastic syndrome, the patient underwent an abdominal ultrasonography, computed tomography and magnetic resonance imaging that showed a single hepatic massive lesion (12x8x7 cm). The biopsy performed was compatible with cholangiocarcinoma. The patient died of intraparenchymal hemorrhage in the external lenticocapsular region, with no identifiable cause. With this clinical case it is intended to alert about the association between dermatomyositis and solid tumors.