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Intravenous leiomyomatosis (IVL) is a rare smooth muscle neoplasm that is histologically benign but biologically aggressive, arising from uterine leiomyomas or the smooth muscle of uterine vein walls. Its distinguishing characteristic is intraluminal venous extension, in which tumor proliferate within uterine, ovarian or iliac veins and may extend proximally into the inferior vena cava (IVC), right heart and pulmonary arteries, producing a spectrum of clinical manifestations [, , , ]. A systematic review reported cardiac involvement in approximately half of patients and an overall mortality of around 2.4% []. Because early-stage disease often mimics a typical uterine leiomyoma on imaging and symptoms are nonspecific, IVL is frequently misdiagnosed as benign fibroids, gynecologic malignancies, venous thrombosis or metastatic disease [, , ]. Consequently, despite its original description in 1896 [], IVL remains diagnostically and therapeutically challenging, with most available evidence derived from case reports and small series. Complete surgical resection is considered the cornerstone of treatment [,], but its success critically depends on accurate preoperative identification of the full extent of intravascular tumor. Contrast-enhanced CT, MRI, and ultrasound are commonly used for IVL diagnosis; however, systematic assessment of their performance—particularly multimodal image analysis that simultaneously targets both the primary pelvic lesions and intravascular extensions—has yet to be fully elucidated. To address this gap, we conducted a decade-long retrospective study of IVL cases from 2 tertiary centers, analyzing clinical and multimodal imaging data (ultrasound, CT, and MRI). We aimed to characterize typical and atypical imaging features of pelvic and intravascular IVL components, identify causes of misdiagnosis, and develop imaging-guided strategies for complete tumor mapping and surgical planning.