Background Intravascular leiomyomatosis (IVL) is a histologically benign smooth muscle tumor arising from the uterus that can spread through the pelvic veins and can extend as far as the heart via the inferior vena cava. IVL is an extremely rare phenomenon, especially when the heart is involved. However, despite its benign characteristics, it can behave like a malignant tumor leading to significant morbidity and even mortality if left untreated. Case presentation The patient is a 42-year-old woman with a past medical history of uterine leiomyomas. Recently she developed heavy bleeding and frequent spotting; therefore consulted with her gynecologist. After further evaluation, a mass within the uterus that expanded into the pelvic veins, inferior vena cava, and right atrium was discovered. After the complete removal of the mass, the patient underwent full recovery. IVL with cardiac extension was the final diagnosis. Conclusion Although IVL is rare, it must be considered in women who underwent previous hysterectomies or myomectomies and presented with pelvic masses or symptoms of right heart failure. The ideal treatment will require the joint efforts of a complete multidisciplinary team, the patient's symptoms, previous operative history, the tumor's extension, and resectability.