Background: Budd-Chiari syndrome (BCS) is a rare disease de ned as hepatic venous out ow obstruction at any level from the hepatic venules up to the cavo-atrial junction. Transjugular Intrahepatic Portosystemic Shunt (TIPS) is performed as a decompressive treatment in some patients.Aim: To evaluate the potential role of Transient Elastography (TE) in assessing liver stiffness in patients with primary BCS. Methods: Twenty one BCS patients and 10 patients with liver cirrhosis with different underlying etiologies underwent abdominal ultrasound and TE.Results: Ninety-ve percent of BCS patients had liver stiffness compatible with F4 with a median of 21 kPa, values which are usually obtained in patients with liver cirrhosis. Ten BCS and 10 cirrhotic patients underwent repeated TE with a median of 320 days between exams for BCS and 4.5 years for cirrhotic patients. The change of liver stiffness in BCS patients was 5.75 kPa (range − 0.4 to 26.6), compared with − 4.85 kPa (range − 15.6 to 15.0) in cirrhotic patients (p-value = 0.0029). Change in liver stiffness from baseline to follow-up in BCS patients who underwent TIPS (n = 4) was 0.2 kPa (range − 0.4 to 15.3), whereas in patients without intervention (n = 6) it was 6.75 kPa (range 1.3 to 26.6). The difference was not statistically signi cant.Conclusion: Liver stiffness in BCS patients is a dynamic progressive process with parameters of TE resembling liver cirrhosis. Even if TIPS seem to slow down the increment of liver stiffness, because of decreased liver congestion, it kept most patients with high score. The TE in BCS patients may be considered for monitoring for stable or upfront disease deterioration.