A primary leiomyosarcoma (LMS) arising from the left fallopian tube in a perimenopausal 48-year-old woman is reported. Primary tubal LMS is an uncommon, exceedingly rare neoplasm, accounting for only a few reported cases so far. To our knowledge, the present case is the 17th tubal LMS reported in the English-language literature. The diagnosis is usually made at the time of laparotomy for a pelvic or adnexal mass or other gynaecological indications. As in ovarian neoplasms, the mainstay of treatment is represented by debulking surgery consisting of total abdominal hysterectomy, bilateral salpingo-oophorectomy, random biopsies, peritoneal washing and excision of all the abdominal tumour masses. Although the approach is radical, the clinical behaviour is very poor. The role of adjuvant radio- or chemotherapy still remains unsolved.