Primary tumors of the upper urinary tract are rarely reported in the literature. We thus report here four cases of primary tumors of the upper urinary tract that were difficult to diagnose, in three women and one man. Preoperative diagnosis was established in only two cases requiring a nephro-ureterectomy. The other two cases were surprisingly diagnosed during anatomopatho- logical examination. Preoperative diagnosis was a pyonephrosis in one case and a renal failure resulting from stenosis of the pelvic ureter in the other. Despite modern methods, primary tumors of the upper urinary tract remain difficult to diagnose.