{Reference Type}: Review {Title}: [A rare malignant tumor of the renal vein]. {Author}: Mouaddine A;Thiebaut PA;Sabourin JC; {Journal}: Ann Pathol {Volume}: 44 {Issue}: 2 {Year}: 2024 Mar {Factor}: 0.411 {DOI}: 10.1016/j.annpat.2023.11.001 {Abstract}: The renal vein is an exceptional location for leiomyosarcoma, an aggressive malignant tumor of smooth-muscle origin with a poor prognosis. We report the case of a 55-year-old female patient who consulted for left flank pain that had been present for 6 months. A CT scan revealed a 9.4cm left retroperitoneal mass in contact with the psoas muscle, left kidney, stomach, spleen, left colon and extending up to the pancreas, raising the suspicion of a tumour originating in the retroperitoneal tissues. A biopsy revealed a smooth-muscle cell tumour with a degree of malignancy difficult to define. The patient underwent a monobloc left compartmentectomy, which led to the diagnosis of leiomyosarcoma of the left renal vein. A review of the literature on these rare tumours in this location is presented.