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Background and Aim: Behçet's syndrome (BS) is a known cause of Budd-Chiari syndrome (BCS). We aimed at identifying the prevalence of BS in patients with BCS, analyzing different clinical presentations, treatment modalities and outcome of these patients., Methods: We conducted a retrospective cohort study, in which all medical records of patients who were presented to Tropical Medicine Department, Ain Shams University with a confirmed diagnosis of primary BCS from May 2005 to December 2016 were collected and analyzed., Results: In total, 271 patients had a confirmed diagnosis of primary BCS, included Group I: 232 (85.6%) patients with BCS without BS and Group II: 39 patients (14.4%) with BCS due to BS. Male gender (P=0.000), oral ulcers, genital ulcers, Prominent abdominal veins, lower limb swellings, lower extremity deep venous thrombosis (P=0.000) and jaundice (P=0.003) were more frequent in group II patients. The presence of intrahepatic collaterals (P=0.004) and IVC thrombosis (P=0.000) was significant in group II. Medical treatment alone in the form of immunosuppressive drugs and anticoagulation (66.7% vs. 24.1%)±IVC stenting (23% vs. 1.3%) (P=0.000) were the main treatment modalities for BCS related to BS. The frequency of HCC in BS was significantly higher (10.26% vs. 2.59%) (P=0.013)., Conclusions: The prevalence of BS in Egyptian patients with BCS is considerably high. The clinical presentation of these patients was different from those without BS. Besides, the incidence of HCC was higher in patients with BS, whereas the mortality did not differ between the two groups., (Copyright © 2019 Elsevier Masson SAS. All rights reserved.)