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Tubulocystic carcinoma of the kidney is a rare entity, which are with about 100 cases of these tumors of the kidney all over the world. We have reported a case, of a 43-year-old man who presented with a lump in the right kidney on image examination without other complaints. Final pathologic diagnosis was tubulocystic carcinoma of the kidney with multiple focal clear cells. Six months after the procedure, the patient remained free of disease. This case showed an indolent manner a low-grade malignancy with favorable prognosis in a short-time follow-up.
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