RESUMO
Although glomus tumour is usually seen in the subungual region of the extremities, it may rarely occur in visceral organs. Approximately 1% of all glomus tumours are malignant. Malignant glomus tumours of the urinary system are extremely rare. This paper presents a case of malignant glomus tumour of the left ureter in a 41-year male patient who had undergone a simple left nephrectomy 4 years ago and presented with recurrent macroscopic haematuria. Uretectomy and mass excision were performed. No progression was observed during the 6-month post-operative follow-up. The tumour can be differentiated from other tumours by pathological and immunohistochemical examination following surgical excision. Because there are very few cases, the treatment protocol is not entirely clear. However, complete removal of the tumour is an effective treatment and can prevent local recurrence. Key Words: Malignant glomus tumour, Ureter, Treatment, Urogenital system.