Abstract
Upper tract urothelial carcinoma represents a small proportion (5%-10%) of all urothelial cancers. Although there are several management options, in undifferentiated or high-risk cases, surgery in the form of nephroureterectomy is the gold standard. Horseshoe kidney is the most common congenital renal fusion anomaly affecting 1 in 400-600 patients. We present the case of a smoker in her mid-50s with an incidental finding of a papillary lesion in the right renal pelvis of her horseshoe kidney on CT scan. She went on to have endoscopic assessment confirming no other foci of disease. She was definitively managed with a robotic nephroureterectomy.