Abstract: Objective To study the levels of tumor necrosis factor-α (TNF-α) and nucear factor-κB (NF-κB) in serum of patients with Budd-Chiari syndrome (BCS), and to explore whether serum TNF-α and NF-κB play an important roles in the process of simple BCS into congestive cirrhosis and liver cancer. Methods There were 103 cases of Budd-Chiari syndrome in the experimental group. During the same period, 130 controls were recruited from the health examination center. The levels of serum TNF-α and NF-κB were tested by ELISA. Results The serum concentrations of TNF-α and NF-κB in the experimental group were higher than those in the control group( P＜0.001). In the BCS group, the serum concentrations of TNF-α and NF-κB were different significantly between pure BCS patients, BCS with stasis cirrhosis patients and BCS with hepatic carcinoma patients( P＜0.001). Among them, the serum concentrations of the two factors of BCS with hepatic carcinoma patients were higher than BCS with stasis cirrhosis patients, and the latter was higher than the pure BCS patients( P＜0.05). Serum TNF-α and NF-κB were positively correlated in the BCS group (r = 0.8, P <0.001). Conclusion The expression level of TNF-α and NF-κB increase in the process of pure BCS deteriorating into congestive liver cirrhosis and even liver cancer. The combination of interventional therapy and anti－fibrosis therapy at an early stage may reduce the incidence of congestive liver cirrhosis and even liver cancer.