Mucinous tubular and spindle cell carcinoma is a rare and newly described type of renal cell carcinoma (RCC) with a relatively indolent behaviour. We report a case of 53-year-old woman who had macroscopic hematuria and flank pain. A right radical nephrectomy was performed showing a well circumscribed tumour confined to the kidney and measuring 17cm. The histologic findings consisted of tightly packed, small, elongated tubules separated by pale mucinous stroma. The tumour cells were cuboidal to spindled with eosinophilic cytoplasm and lower nuclear grade. Tumour was immunoreactive for cytokeratin CK 7, CK19 and epithelial membrane antigen (EMA). The morphological and immunohistochemical features were consistent with mucinous tubular and spindle cell carcinoma of kidney.