Abstract
Uterine leiomyomas are common, benign neoplasms of the uterine smooth muscle. Leiomyomatosis is uncommon and causes development of multiple leiomyomas that can manifest as intravascular leiomyomatosis (IVL). We present the case of a 46-year-old female with IVL extending from the right gonadal vein to the right atrium and pulmonary arteries with an independent renal cell carcinoma of the right kidney. She underwent successful open right radical nephrectomy, inferior vena caval tumor thrombectomy and pulmonary embolectomy. While there was initial concern for hereditary renal cell carcinoma, final histologic testing did not support the diagnosis.