The authors report a case of fibro-epithelial polyp in a young woman with Turner syndrome with features of a right ureteropelvic junction syndrome in a horseshoe kidney. The patient presented a history of chronic low back pain associated with macroscopic haematuria leading to various complementary investigations including ureteroscopy. The endoscopic appearance of the tumour suggested the diagnosis despite the presence of atypical renal pelvis cytology. Pyeloplasty and proximal ureterectomy allowed confirmation and treatment of this rare lesion.

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