Renal medullary carcinoma is an aggressive malignant tumour, recently reported in the literature. It is usually reported in the relatively young patients with drepanocytic trai. Histologically, the tumour is constituted by a tumoral proliferation with diffuse or glandular architecture and inflammatory stroma. The carcinomatous cells have plasmocytoid or rhabdoid aspect. We report a case of 40 years old man who presented macroscopic hematuria. Through this observation and the review of the literature we discuss the anatomoclinical and the prognostic aspects of this exceptional tumour.