Primary development of malignant mixed Müllerian tumor (MMMT) in the fallopian tube is a very rare pathology associated with negative prognosis. The authors report a new case of Stage III B tubal carcinosarcoma with no heterologous element. After the surgical radical procedure, a combined adjuvant chemotherapy was given and, after two years of follow-up, the patient shows no evidence of disease. A literature review of articles on this topic was conducted through a Medline search for articles published in English, and 63 patients with the same pathology were found. Data on symptoms, diagnosis, histological type, treatment, and prognosis were collected and statistically analyzed. As conclusion, the initial stages have better survival, with no effect of histological subtype. Presence of ascites had a negative prognostic value. Due to the limited number of cases reported, making the statistical analysis difficult, the authors consider mandatory to report this rare entity to ease the upcoming research.