Dermatomyositis is a chronic inflammatory disease involving the skin and muscles. It most commonly occurs in adults with preponderance in females, but pediatric occurrence is also possible. The risk of malignancy in adult patients with dermatomyositis was reported to be 4.66-fold higher compared to that in the general population. A significantly increased risk of malignancy was reported within the first 12 months following the diagnosis of dermatomyositis (standardized incidence ratio equaled 17). One of the characteristic laboratory findings associated with dermatomyositis is the presence of circulating autoantibodies which are classified into two subgroups: myositis-specific and myositis-associated autoantibodies. It was shown that specific types of antibodies might be associated with an increased risk of malignancy. Current literature data indicate that the strongest correlation with malignant diseases was reported in anti-TIF1-γ-positive patients who were at a 9.37-fold higher risk of cancer. A 3.68-fold increase in the risk of cancer was also reported among patients with anti-NXP2 antibodies. Malignant diseases were reported in 14–57% of patients with anti-SAE antibodies. The presence of other autoantibodies may also be associated with an increased risk of malignancy. These data indicate that patients with circulating anti-TIF1-γ, anti-NXP2, and anti-SAE should be very closely monitored for dermatomyositis-associated malignant comorbidities. The aim of this review is to summarize the current data regarding the link between malignancy and the presence of specific antibodies in patients with dermatomyositis.