Introduction: Budd-Chiari Syndrome is a rare entity that is defined as partial or complete obstruction to the venous outflow of the liver. It is classified as primary, when the obstruction is intra-luminal, or secondary if it is due to extrinsic vascular compression or tumor invasion. The clinical presentation is variable, and depends on the extent and speed of installation, being able to manifest in an acute, subacute or chronic form. Objec- tives: To describe the epidemiological characteristics, clinical presentation, etiology, treatment and evolution of Budd-Chiari Syndrome in the cohort of patients assisted in the National Liver Transplant Program of Uruguay. Materials and Methods: Retrospective, descriptive study, carried out in the period between January 2014 and December 2019. Results: Of the 7 enrolled patients, the female gender predominated (85%). The median age was 31 years, with an interquartile range of 19 years. 85% of the patients presented with compli- cations related to portal hypertension, and one case as acute liver failure. The MELD-Na at diagnosis was 15, with an interquartile range of 10. In relation to the etiology, all were of primary cause and in 6 an underlying prothrombotic state was diagnosed. A multimodal and staggered therapeutic approach was used. All patients received anticoagulant treatment, and in 2 of them no additional therapeutic measures were necessary. Those who maintained clinical elements of portal hypertension, or clinical manifestations that suggested poor response to treatment, progressed in the therapeutic algorithm, with TIPS in one patient and liver transplantation in 4. Survival at the end of the study was 100%, after 24-month follow-up, with an interquartile range of 38 months. Conclusions: A descriptive analysis of a group of patients with Budd-Chiari Syndrome was carried out, noting characteristics similar to those described in other series. Although it is a small sample, it is the largest series described in Uruguay and is close to other reports from the region. The staggered therapeutic approach allowed the survival of all patients.