Angiosarcoma arising in the retroperitoneal space is rare. We report a case of perirenal angiosarcoma presenting preoperative diagnostic difficulties. A 49-year-old man was referred to our department with left kidney mass. Specimens of CT-guided percutaneous needle biopsy of the mass did not contain any atypical cells suggestive of malignancy. A CT scan 6 months after embolization of the tumor revealed growth of the mass and two space-occupying lesions appearing in the liver. Laparoscopic resection of the left kidney with perirenal mass and one of the liver lesions was performed. Histopathological findings confirmed a diagnosis of perirenal angiosarcoma with hepatic metastases.