Leiomyosarcomas of the inferior vena cava are rare and the clinical symptoms unspecific. We report a case of leiomyosarcoma of the inferior vena cava in an 82-year-old woman presenting with weight loss and abdominal pain. Following elaborate preoperative examinations, surgical resection was performed and the inferior vena cava was reconstructed. Clinical signs, diagnosis, therapy, and prognosis are discussed.