Resection of the renal vein leiomyosarcoma with preservation of the kidney

: We present a case of 75 years old woman who successfully underwent resection of the right renal vein leiomyosarcoma (LMS-VR) with preservation of the kidney. It is the first reported case in Polish and fifth in world literature. The patient is alive without recurrence 24 months after operation. The surgical resection of LMS-VR with the preservation of the kidney should always be considered when the tumour does not infiltrate the renal hilus.