Introduction: Renal mucinous tubular and spindle cell carcinomas are uncommon, distinctive renal tumors accounts for less than one percent of renal cell carcinoma. Because of such a rare and important presentation, we report a mucinous tubular and spindle cell carcinoma of kidney which occurred in a 60-year-old woman. Case Presentation: A 60-year-old woman presented complaining of vague epigastric pain since 2 months. She did not have any urinary problems such as haematuria or loin pain. Contrast enhanced CT scan of the abdomen and pelvis detected a large left renal mass lesion (11 × 9 cm in maximum dimensions) arising from middle zone of the left kidney. The lesion shows heterogenous contrast enactment with radiologic criteria suggestive of aggressive neoplastic lesion (Renal cell carcinoma). Left radical nephrectomy was done through flank incision. The histopathology examination reported mucinous tubular and spindle cell carcinoma. After 4 months, a contrast enhanced CT scan of the abdomen and pelvis was performed. No detectable local or distant recurrence seen. Right kidney was of normal appearance and excretory function. Conclusion: Radical nephrectomy is the preferred treatment for this tumor as it has a favorable prognosis. However, a small percentage of cases may experience recurrence or metastasis, necessitating regular follow-up.
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