Mucinous tubular and spindle cell carcinoma (MTSCC) of the kidney is an uncommon recently recognized renal cell carcinoma. We reported A 60 year's old man who presented with right flank pain, abdominal swelling and one attack of hematuria. The intraoperative finding was a huge cystic swelling arising from the right kidney occupying almost all the abdominal cavity displacing the bowel to the left side of the abdomen. There was no ascites or evidences of metastasis. Right radical nephrectomy was done . Then the diagnosis of renal MTSCC was established. General condition of the patient was improved and one year prognosis was satisfactory. This is the first reported case of MTSCC in Sudan, and the outcome of treatment was satisfactory .