Squamous cell carcinoma, originating in the renal pelvis, is an infrequent form of kidney malignancy. The occurrence rate remains below 1% for all neoplasms in this specific area. Most of these carcinomas are moderately or poorly differentiated, and diagnosis typically occurs at an advanced stage. Xanthogranulomatous pyelonephritis is an uncommon form of severe chronic infection that affects the parenchyma of native kidneys. We present the case of a 34-year-old male with a history of end-stage renal disease secondary to recurrent pyelonephritis, which was incidentally diagnosed as renal squamous cell carcinoma (SCC).