Primary venous leiomyosarcoma is very rare and first description was made by Perl in 1871. Most cases are from venous system and half of them are reported to originate in inferior vena cava. We report the case of a 77-year-old Caucasian man with a leiomyosarcoma of the femoral vein. A leg preservation surgery was decided. Eight months later, the patient died of tumor progression. A PubMed search using the terms "leiomyosarcoma femoral vein" and "leiomyosarcoma vein" was performed. All cases of composite iliac-femoral leiomyosarcoma were excluded, and as far as possible, only well-documented cases were included. Median age was 55 years, seven men and six women, tumor resection was performed by six authors, six others performed a vascular resection, and one preferred for a thigh excision. Prognosis remains poor if metastasis is present, and in these cases, a conservative surgery is recommended to preserve patients' quality of life.
Keywords: Femoral leiomyosarcoma; Femoral vein; Leiomyosarcoma; Surgery.