Background and objectives: Budd–Chiari syndrome is a vascular disorder of the liver which can cause fulminant liver injury and lethal portal hypertension-related complications. It is a rare disease and can be primary or secondary. The objective of our work is to detail the etiologies, treatment and evolution of SBC according to the experience of a Moroccan center. Patients and methods: This is a retrospective and descriptive study in the university hepato-gastroenterology department including all patients with BCS with portal hypertension (PH) over a period of 29 years. All our patients benefited from an etiological work-up and morphological explorations. Results: Out of a total of 364 cases of vascular liver disease, 29 patients had BCS, with a prevalence of 8%. Clinically, the signs of decompensated PH were predominant. Imaging confirmed BCS. The etiological work-up showed Behçet’s disease in 17%. In one case each, BCS was secondary in 6.8% of cases (n=2), one of whom had HCC and the second had an association of sarcoidosis and amyloidosis. Our patients had received treatment for the causative disease and treatment of thrombosis associated with the treatment of PH complications. The evolution was marked by the death of 5 patients (17%). Conclusion: Budd-Chiari syndrome (BCS) is a rare condition. Behcet ' s disease and thrombophilia are the most frequent etiologies in our series. The prognosis is poor, mainly due to the causative disease and complications of PH, which requires a very early management.