Introduction <p>Intravenous leiomyomatosis (IVL) is rare and is characterized by the extension of an aggressive, histologically benign tumor into venous channels and cardiac chambers. We report a case of advanced IVL to highlight its insidious presentation and the requisite multidisciplinary surgical approach.</p> Case Presentation <p>A patient presenting with nonspecific symptoms was incidentally diagnosed with stage III IVL with intracardiac extension. A multidisciplinary team performed a single-stage procedure that involved a total hysterectomy with bilateral adnexectomy and left ovarian vein resection, followed by right atrial tumor resection under extracorporeal circulation (without cardiac arrest), IVC tumor removal, and resection of a left renal vein tumor embolus. The surgery was successful, with an uneventful recovery.</p> Conclusions <p>This case highlights that IVL can be asymptomatic until it reaches an advanced stage. A meticulous, single-stage multidisciplinary surgical strategy is feasible and effective for complete tumor resection, even in the presence of intracardiac involvement, ensuring optimal patient outcomes.</p>