Kidney leiomyosarcoma represents a rare variety of malignant kidney tumours. In this paper, we report on a patient with an inoperable leiomyosarcoma. Since this neoplasm is very rare, there is very little information on this type of malignancy. We present the symptoms, radiological findings, diagnostic criteria and differential diagnosis of the tumour. Leiomyosarcoma exhibits aggressive biological behaviour and has a poor prognosis. We have found that the treatment of choice is a radical nephrectomy.

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