Inferior vena cava (IVC) leiomyosarcoma is a rare malignant neoplasm more commonly seen in women in the fifth to sixth decade of life. Complete resection of the tumor with negative margins is the only therapeutical option that has demonstrated a survival benefit. This report presents a case of a 67-year-old woman affected by a lower segment IVC leiomyosarcoma incidentally detected during a chronic abdominal pain study. The patient was treated with tumorectomy, resection and ligation of the infrarenal IVC without signs or recurrence on a 12-month follow-up.