A case of a 30-year-old female patient diagnosed with mucinous tubular and spindle cell carcinoma (MT&SCC) of her left kidney of the basis of an abdominal ultrasound scan performed when nonspecific pain is presented. The histopathology report revealed a classic type of MT&SCC. Photographs of histopathological samples are shown. Nephrectomy was applied as sole treatment. A follow-up of six years confirmed surgery to be a sufficient approach. The discussion presents a review of up to date world literature concerning the histopathological features, prognosis, and treatment of MT&SCC.