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Mucinous adenocarcinoma of the renal pelvis is a rare disease and preoperative diagnosis is difficult. It often presents with a mucinous-like discharge and is associated with prolonged irritation of inflammation or infection. We present a case in which the patient suffered from right flank dull pain with staghorn stone, and share our clinical experience of renal pelvis mucinous adenocarcinoma. The patient underwent right nephroureterectomy and pathological stage was pT1. He did not receive adjuvant chemotherapy. No recurrent tumor or metastasis was noted for about 1 year.