The occurrence of primary ureteral carcinoma of the remnant ureter after nephrectomy for benign disease is very rare, and such cases in a kidney transplanted patient are even rarer. The case of a 62-year-old female patient is presented who had end stage autosomal dominant polycystic kidney disease and in 1991 underwent cadaveric kidney transplantation. Immunosuppression therapy was maintained with evorolimus and prednisolone. Normal serum creatinine levels were maintained and no signs of allograft rejection were detected. In 2010, the patient developed intermittent macroscopic haematuria and was admitted for further evaluation. Computer tomography, cystoscopy, laboratory tests and urine cytology were performed and no apparent reason for the haematuria was found. After more episodes of uncontrollable haematuria, in 2011 she underwent bilateral nephrectomy of her native kidneys. In 2015, after another episode of haematuria, a CT was performed on the patient. She was diagnosed with a solid enhancing mass in the right ureteral stump. Ureterectomy with bladder cuff excision was performed with lower midline laparotomy. The specimen was verified as high grade urothelial carcinoma, in stage T1 according to the 2004 WHO grading system. To the best of the authors’ knowledge, this is first case in the availabe literature of the occurrence of transitional cell carcinoma developing in the remnant ureter in a kidney transplanted patient after bilateral prophylactic nephrectomy for uncontrollable haematuria – 24 years after transplantation.