Abstract: Objective To investigate the clinicopathologic characteristics, histogenesis, differential diagnosis and prognosis of Mucinous Tubular and Spindle Cell Renal Cell Carcinoma (MTSC). Methods Two cases MTSC were examined by HE, histochemical and immunohistochemical stains, followed up for 39 and 83 months, and the related literatures were reviewed. Results The patients were both females, at ages of 60 and 73. The tumors were well circumscribed. Tumors consisted of tightly packed, small, elongated tubules separated by pale mucinous stroma. The tumor cells were cuboidal to spindled with eosinophilic cytoplasm and low nuclear grade. Mitoses were few or nonexistent. Both were immunoreactive for CK34βE12, E-cadherin, CK7, CK19, vimentin and one case was partly positive for NSE and synaptophysin. The proliferative rate (Ki-67) is <5%. Prognosis was favorable in our two cases. Conclusion MTSCs are rare and low-grade renal epithelial neoplasms. immunohistochemistry favored the hypothesis of a distal tubule origin partly with neuroendocrine differentiation, relatively similar to papillary renal cell carcinoma. Radical nephrectomy alone was needed, without adjuvant therapy, but Follow-up in the future is necessarily.