Only a few cases of leiomyoma of the vena cava or iliac vein and, according to our knowledge, only one case of renal vein leiomyoma have been reported. We report a patient with leiomyoma of the left renal vein. Tumour resection was performed by resecting a part of the vein along with the tumour and by ligation of the vein. Left kidney drainage was established through the preserved ovarian vein. In order to establish a diagnosis, careful pathologic examination of multiple sections has to be done and because of the potentially malignant behaviour, long-term follow-up after total resection is necessary.