Dermatomyositis is well known to be associated with several types of malignancy and patients with dermatomyositis have higher rates of mortality from cancer. Although rare in Japan, head and neck cancer, especially nasopharyngeal cancer, is the predominant type of cancer associated with dermatomyositis in several areas in Asia, including Hong Kong and Singapore. Here we report two cases of head and neck cancer with dermatomyositis as well as a literature review. Both cases were treated with chemotherapy and radiotherapy. Although the patients were immunosuppressed due to dermatomyositis treatment, no grade 3 or 4 adverse events occurred.