Leiomyosarcoma affecting the renal vein is rare, with about 30 documented cases in the English literature. The appearance on computed tomography can be difficult to interpret and is often confused with advanced renal cell carcinoma (RCC). This confusion can have implications on the perioperative care of patients presenting with this disease. We report a case with an usual radiological appearance of a renal vein leiomyosarcoma, alongside a separate RCC. This case highlights the need for a high index of suspicion in radiological reporting and provides a dilemma in regards to postoperative surveillance.