A 68-year old man presented with erythemato-telangiectatic nodules on his right lumbar fossa, associated with right-sciatica. A skin biopsy was performed. Microscopic examination showed tumour embolism in the lumen of the dermal vessels. Immunohistochemical staining and electron microscopy confirmed the diagnosis of angiosarcoma metastasis. An arteriography showed the primary neoplasm as a polypoid tumour with anterior pedicle, narrowing the aortic lumen below the renal arteries. The patient died a few months later, with multiple metastasis in the lower half on his body. To our knowledge, this is the twelfth case of arterial primary angiosarcoma reported in the literature and the fifth one with skin metastasis. The previously published cases are reviewed.