Primary leiomyosarcoma (LMS) of the renal vein is a rare tumour and poorly described in the literature. Surgical resection, using open and laparoscopic approaches, is the mainstay of treatment. In this report, we describe a patient with left renal vein LMS, report the first robotic laparoscopic resection for this tumor, and review the typical presentation, imaging, pathology and treatment for this rare clinical entity.