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Introduction Budd-Chiari syndrome (BCS) is a vascular liver disorder that can lead to lethal portal hypertension (PH) complications. It is a rare disease that can be either primary or secondary. This severe condition is caused by the obstruction of the hepatic veins up to the terminal part of the inferior vena cava. Diagnosis relies on imaging, notably abdominal Doppler ultrasound. Treatment may include addressing the underlying cause, managing complications, and alleviating symptoms.The objective of this work is to analyze the clinical presentation, paraclinical findings, complications, treatment, and evolution of BCS.Method This is a retrospective and descriptive study including all patients with BCS over a period of 30 years, from 1995 to 2025.Results In our study on BCS, which involved 34 patients, ages ranged from 12 to 64 years, with a mean age of 31 years and a sex ratio of 4.5 F/ M. Jaundice was the most common medical history, present in 12% (n = 12) of cases. Diagnosis occurred within the same year of symptom onset in 73% (n = 25) of patients. The predominant symptom was abdominal distension, observed in 48% (n = 16) of cases. Clinical examination revealed the predominance of signs of PH, notably collateral venous circulation 73% (n = 25), ascites in 55% (n = 19), and splenomegaly in 44% (n = 15). The most frequent complication was ascites, present in 67% (n = 17) of cases. Imaging confirmed BCS in all patients. The liver function tests showed cholestasis in 73% (n = 25), liver cell failure in 47% (n = 116), and cytolysis in 35% (n = 12) of cases. Primary causes predominated, including Thrombophilia in 27% (n = 9), Behcet in 14% (n = 5) and Myeloproliferative Neoplasms (MPN) in 12% (n = 4) of cases. Secondary causes were found in 2 patients, namely hepatocellular carcinoma and sarcoidosis. All patients had received etiological and symptomatic treatment, as well as treatment for thrombosis associated with the treatment of PH complications. The outcome was marked by the death of 5 patients (15%).Conclusion Our study highlights the complexity and severity of BCS. Despite timely diagnosis and treatment strategies, the disease still posed significant challenges. The predominance of primary causes underscores the importance of thorough clinical evaluation and targeted therapy. The high prevalence of complications and the significant mortality rate of 15% emphasize the need for ongoing research and improved management approaches to enhance patient outcomes in BCS.