Here we describe the clinical, radiologic, histopathological and immunohistochemical features of a rare spontaneous rupture of renal angiosarcoma detected in the left kidney of a 55-year-old male. Due to the rarity of this neoplasm (less than 15 cases have been reported), there is no unanimous concensus for therapy following radical nephrectomy. In our case there was no flank trauma and retroperitoneal haematoma around the left kidney was seen on computed tomography. The patient underwent left radical nephrectomy and died about 3 months after operation due to multiple bone and liver metastases. To our knowledge, this is the first report of spontaneous rupture of renal angiosarcoma and we suggest that it should be borne in mind that renal angiosarcoma may be a cause of retroperitoneal haematomas.

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