Patients with dermatomyositis may face an increased risk of malignancy. Malignant melanoma has been linked to dermatomyositis. We present a case of concomitant dermatoyositis and metastatic malignant melanoma and review the English literature concerning this topic. Analysis revealed that the development of dermatomyositis in patients with malignant melanoma heralds the diagnosis of metastatic disease. These patients face dismal prognoses.