Dermatomyositis, an idiopathic inflammatory myopathy with characteristic skin manifestations, is associated with several types of cancer. Only three cases of gallbladder cancer with dermatomyositis have been reported worldwide, and none has been reported in Korea. We present a case of a 71-year-old male with proven dermatomyositis associated with gallbladder cancer to emphasize the necessity of work-up for malignancies with low incidence in patients with inflammatory myopathies.