Dermatomyositis is a rare idiopathic inflammatory myopathy characterized by pathognomic cutaneous manifestations (heliotrope rash, periorbital oedema, and Gottron’s papules) and proximal muscle weakness. Approximately one third of the patients experience paraneoplastic dermatomyositis. In Western populations, the most frequent underlying malignant disease is ovarian, breast, lung, pancreatic, colon cancer, as well as non-Hodgkin lymphoma. The pathogenesis of the disease is not completely clarified. However, it is considered that the antigens expressed on the membranes of malignant cells initiate the forming of autoantibodies, which then tie to muscle fibres and cause myositis. Corticosteroids represent the mainstay treatment, with immunosuppressants as a frequent addition. Although it is the most frequent biliary duct cancer, gallbladder cancer is a rare malignant disease. Several risk factors are recognized, as well as several carcinogenesis theories. Due to long asymptomatic course and the absence of adequate selection, the disease is mostly diagnosed in locally advanced and metastatic stage. Therefore, the prognosis is poor. In this paper, the case of a 48-year-old female patient whose dermatomyositis was initially diagnosed as vasculitis is presented. Following the patient's inadequate response to corticosteroid treatment, clinical and radiologic examinations were performed, showing inoperable gallbladder adenocarcinoma. Although initial chemotherapy led to regression, the dermatomyositis developed an independent course with new pathological changes leading to the progression of the disease. Besides presenting the case, in January 2019, PubMed database was searched by using MeSH terms gallbladder neoplasm and dermatomyositis. A total of nine case reports with dermatomyositis and gallbladder cancer were found (seven females, including the patient presented here, and two males). A surgical procedure was undertaken on two patients, after which the resolution of cutaneous manifestations followed. Three patients were given chemotherapy. Although in the last decade a progress in comprehending both diseases was made, the cooperation of specialists in various fields is still necessary. In patients with long-term gall bladder cancer risk factors, abdominal US and consulting on prophylactic cholecystectomy should be obligatory procedures. In patients with dermatomyositis, gastrointestinal (gallbladder, pancreatic, and colon) carcinoma should not be omitted, so US should be performed.