Upper tract urothelial carcinoma (UC) is an uncommon tumor. Ectopic kidney is also a rare entity. The combination of these two conditions is very rare. A 49-year-old male complained of right flank pain with hematuria. On CT scan he was found to have a malrotated right kidney with soft tissue seen in the upper calyceal group and a normal left kidney. Diagnostic cystoscopy was unremarkable. Radical nephroureterectomy with bladder cuff excision was performed. Pathology report revealed low grade urothelial carcinoma. Patient's symptoms disappeared postoperatively. Follow up showed no recurrence during the first two years in the bladder and upper tract in the contralateral kidney. Isolated UC of ectopic kidney is rare disease three cases were reported in literature. Although treatment of this tumor can be challenging due to its complex blood supply and position inside the pelvis, treatment strategy is still similar as for orthotopic kidneys.

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