Primary cutaneous carcinosarcoma is a biphasic tumour containing both malignant epithelial and malignant mesenchymal elements. To date, only 26 cases have been reported in the literature. However, our findings suggest that this may reflect underreporting and possibly underdiagnosis. We present five cases and a comprehensive review of the literature: The disease most commonly presents in the eighth and ninth decades of life, is twice as common in males and may be related to sun exposure. Surgery is the primary therapeutic modality. Despite treatment, 27% of cases developed metastatic disease. Both epithelial and mesenchymal elements have been implicated in disease spread. We hope to raise awareness of this uncommon but serious disease and to assist clinicians in its management.