The authors report the fourth case of inverted papilloma of the ureteropelvic junction since the first description of this lesion in 1963 by Potts and Hirst. In view of the rarity of this lesion and its unusual presentation, they conducted an exhaustive review of the literature concerning inverted papillomas of the upper urinary tract. This proliferative lesion of the urothelium can precede, accompany or follow superficial or invasive urothelial tumours. Rigorous treatment and surveillance are therefore justified to detect an associated tumour. The controversy concerning the benign nature of inverted papilloma has still not been clarified.