Introduction: Ureteral ectopia is a rarely observed anomaly. It may be totally asymptomatic. An association with a duplex system is exceptional. Diagnostic and therapeutic approaches are challenging. Carcinologic surgery must consider the anatomic variant, mainly related to the ectopic site of the ureteral orifice.
Observation: We report a case of a ureteral urothelial carcinoma in a North African 52-year-old male patient, in a right duplex system. Radiological explorations concluded a non-functional upper right kidney. A suspect mass was observed in the lumbar part of the ureter of the right upper system. The meatus of the tumorous ureter ended in the right lobe of the prostate. A right hemi-nephro-ureterectomy was performed. A histological examination concluded a pT2G2 urothelial carcinoma.
Conclusion: Even if malignancy is rarely observed in ureteral ectopia, it should be evoked mainly in cases of hematuria with risk factors for urothelial tumors.
Keywords: Nephroureterectomy; Ureter abnormalities; Ureteral neoplasms.