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Purpose: To evaluate the changes in the liver volume and function after direct intrahepatic portocaval shunt (DIPS) in patients with Budd-Chiari syndrome (BCS) with diffuse hepatic vein (HV) occlusion. Methods: The clinical data of 29 patients with BCS who underwent DIPS for intractable ascites caused by diffuse hepatic vein occlusion in the Affiliated Hospital of Xuzhou Medical University were analysed retrospectively; the patients included 8 males and 21 females, with an average age of 33.3 ± 6.3 years. The patients underwent abdominal CT scanning and liver function examinations before DIPS, and 1 week, 3 months, 6 months and 12 months after DIPS. The changes of the liver volume and liver function before and after DIPS were compared. Results: All 29 patients underwent DIPS successfully. 28 patients survived during the follow-up of 12–33 months, with a median follow-up of 16 months. The patients' liver function were significantly improved at 3, 6 and 12 months after the operation compared to before the operation (P < 0.05). The liver volumes measured before the operation and 1 week, 3 months, 6 months and 12 months after the operation were 2124.586 ± 420.889 cm3, 1926.263 ± 372.268 cm3, 1480.592 ± 183.061 cm3, 1461.904 ± 153.027 cm3 and 1469.286 ± 148.549 cm3, respectively. Compared with the preoperative liver volume, the liver volume had decreased significantly at 1 week, 3 months, 6 months and 12 months after the operation (P < 0.05). However, there was no significant difference in the liver volumes at 6 and 12 months after the operation (P = 0.35). Conclusions: Direct intrahepatic portocaval shunt has achieved satisfactory clinical results in the treatment of BCS with diffuse hepatic vein occlusion. The congestive hepatomegaly was gradually reduced after the operation. The liver volume (which was defined as the clinical efficacy in this study) remained stable after 6 months. [ABSTRACT FROM AUTHOR]