A case of primary mucinous carcinoma originating in the renal pelvis associated with renal lithiasis is reported. The left kidney removed was mostly occupied by mucinous carcinoma with areas of signet ring cell carcinoma. The epithelium covering the pelvis consisted of a single layer of columnar epithelium with scattered goblet cells and mucous glands. A series of careful examinations of the abdominal viscera failed to disclose any other primary lesion. The patient died with multiple peritoneal tumor disseminations eight months after the operation despite additional chemotherapy.

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