Transitional cell carcinoma of the kidney with caval tumour thrombus is extremely rare. The authors describe the radiological signs suggesting the preoperative diagnosis and guiding the therapeutic approach. They present the case of a 51-year-old patient with excluded caliceal stones, and review 17 cases published over a period of 24 years during which considerable progress has been made in radiological investigations. Only CT appears to be able to indicate this aetiology and allows retrograde ureteropyelography looking for a tumour of the urinary tract. MRI provides the best assessment of tumour involvement of the inferior vena cava. Nephroureterectomy with excision of a perimeatal bladder cuff and cavotomy is the only oncological surgical procedure, despite the extremely poor prognosis.