•Intravenous leiomyosarcomatosis is a rare condition in which malignant myometrial tissue metastasizes to the heart.•We discuss the complicated diagnosis and treatment of a patient afflicted with intravenous leiomyosarcomatosis.•To ensure a favorable prognosis, therapy should comprise comprehensive and multi-disciplinary treatment.
Keywords: Gynecologic oncology; Intravenous leiomyosarcomatosis; Management.