Abstract
Solitary fibrous tumours (SFTs) are neoplasms originating from mesenchymal cells, usually found in lung pleura. Extrapleuritic lesions are extremely rare, with about 60 cases of SFTs of the kidney available in the English literature. We report here the first case of a malignant SFT of the kidney presenting with an extensive vascular thrombus extending to the right atrium with associated pulmonary emboli. We describe management, pathological assessment, as well as radiological and clinical evolution. Our report provides a first therapeutic approach to a critical presentation of a rare pathology, which should help guide management of such disease in future cases.