Angiosarcoma is an extremely rare vascular malignancy with a 1-year survival rate of 50%, regardless of tumour origin. Distant metastases are common and occur in the lungs, bone, lymph nodes and soft tissues. The majority of patients with angiosarcoma present with localised disease, although 25-45% have distant metastases at presentation. There are few reported cases of angiosarcomas of the bladder and we report the second case in the literature of primary angiosarcoma of the ureter. We suggest that, in atypical manifestations of suspected urothelial malignancy, the multidisciplinary team considers the possibility of rarer, aggressive tumours early. This may influence clinical decision making towards offering radical treatments earlier, before it is too late to do so owing to aggressive oncological behaviour.