Angiosarcoma is the most common primary malignant tumour of the heart. It is a rare and aggressive neoplasm that almost always has a short and fatal evolution. By the time it produces symptoms it has usually progressed to a mass causing haemodynamic compromise. Initial presentation with metastatic disease is unusual. We report the case of a 72-year-old man who presented with painful skin lesions on both hands. The skin biopsy was diagnosed as intravascular metastasis of epithelioid angiosarcoma. Body computed tomography scan disclosed a solid mass in the left atrium. The tumour was judged unresectable and the patient was treated with systemic chemotherapy, consisting of liposomal doxorubicin, which resulted in a complete clinical response. The patient remains free of disease after 48 months of follow-up. The excellent clinical evolution of our patient verifies that liposomal doxorubicin may be effective in the treatment of these tumours and significantly prolong patients’ lifespan.