ObjectiveIntravenous leiomyomatosis (IVL) is a rare disease, and few studies have focused on the diagnostic value of contrast-enhanced ultrasound (CEUS) in this condition. This study aimed to investigate the diagnostic value of CEUS in IVL and summarize the specific CEUS characteristics of IVL.Materials and MethodFrom December 2016 to March 2021, 93 patients admitted to our hospital with inferior vena cava (IVC) occupying lesions were prospectively enrolled and underwent detailed ultrasound multi-modality examinations, including conventional and contrast-enhanced ultrasound scans. The diagnostic value of CEUS and conventional ultrasound (CU) in IVL was compared, and the specific IVL signs were summarized.ResultsAmong the 93 patients with inferior vena cava mass, 67 were IVL while 26 were non-IVL. The inter-observer agreement of the two senior doctors was good, with Kappa coefficient = 0.71 (95% CI: 0.572–0.885). The area under the ROC curve of CU for IVL diagnosis was 0.652 (95% CI: 0.528–0.776), and its sensitivity, specificity, accuracy, positive predictive value, negative predictive value, missed diagnosis rate, and misdiagnosis rate were 61.1%, 69.2%, 63.4%, 83.7%, 40.9%, 38.8%, and 30.8%, respectively. The area under curve (AUC) for IVL diagnosis by CEUS was 0.807 (95% CI: 0.701–0.911), and the sensitivity, specificity, accuracy, positive predictive value, negative predictive value, missed diagnosis rate, and misdiagnosis rate were 82.0%, 84.6%, 82.8%, 93.2%, 64.7%, 15.4%, and 17.9%, respectively. In CEUS mode, “sieve hole sign” and “multi-track sign” were detected in 57 lesions, and the detected rate was higher than that of CU (https://loop.frontiersin.org/people/1014187 < 0.01).ConclusionCEUS can better show the fine blood flow inside the IVL, which is important for IVL differential diagnosis. Moreover, CEUS can obtain more information about IVL diagnosis than CU, compensating for the shortcomings of CU in detecting more blood flow within the lesion. Thus, this technique has great significance for IVL diagnosis.